CLINICAL APPROACH TO ISOLATED SPLENOMEGALY1

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CLINICAL APPROACH TO ISOLATED SPLENOMEGALY : 

CLINICAL APPROACH TO ISOLATED SPLENOMEGALY Prepared by : Dr.Vipin

Introduction : 

Introduction Splenomegaly may be a presenting or dominant feature in a variety of diseases that can interfere with normal splenic function. Good history and careful physical examination. Well defined laboratory studies. Systemic approach

SPLEEN : 

SPLEEN Spleen is a reticuloendothelial organ that arises in the dorsal mesogastrium at about 5 weeks gestation. Spleen becomes enlarged in many disease processes the aetiology ranging from infections to neoplasms such as CML . we are discussing the causes of isolated splenomegaly and clinical approach towards managing it

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The weight of the normal spleen is 75-250 g. . In normal individuals this organ measures about 125 × 75 × 25 mm (5 × 3 × 1 inches) in size It lies in the left hypochondrium between the gastric fundus & left hemidiaphragm .Its long axis lying along 10th rib .there is a notch on it’s inferolateral border which can be palpated when spleen is enlarged. The tortuous splenic artery arises from the coelic axis and runs along the upper border of the body and thje tail of pancreas, to which it gives small branches . the short gastric and left gastroepiploic branches pass between the layers of the gastrosplenic ligament .the main splenic artery generally divides into superior and inferior branches , which in turn, subdivide into many segmental branches. The splenic vein joins superior mesenteric vein at the neck of pancreas to form portal vein.

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red pulp * "sinuses" (or "sinusoids") which are filled with blood* "splenic cords" of reticular fibers* "marginal zone" bordering on white pulp Mechanical filtration. Removes unwanted materials from the blood, including senescent red blood cells. white pulp Composed of nodules, called Malpighian corpuscles. These are composed of:* "lymphoid follicles" (or "follicles"), rich in B-lymphocytes* "periarteriolar lymphoid sheaths" (PALS), rich in T-lymphocytes Helps fight infections.

Functions of spleen : 

Functions of spleen Immune function : spleen processes foreign antigen and is the major site of specific IgM production Filter function : macrophages in the reticulum capture cellular and non-cellular material from blood and plasma . this will include removal of effete platelets and RBCs .This process take place in the sinuses and splenic cords by the action of endothelial macrophages Pitting : particulate inclusions from red cells are removed and the repaired red cells are returned to the circulation. These include Howell-jolly bodies & Heinz bodies , which represents nuclear remnants & precipitated Hb or globin subunits respectively.

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Reservoir function : this function is less marked than in other species, but the spleen does contain app. 8% of the red cell mass .An enlarged spleen may contain a much larger proportion of blood volume . Cytopoiesis : from 4th month of I.U. life ,some form of haemopoiesis occurs in human spleen. Stimulation of the white pulp may occur following antigenic challenge, resulting in the proliferation of T & B – cells and macrophages . This may also occur in myeloproliferative disorders , thalassaemias and chronic haemolytic anaemias .

SPLENOMEGALY : CLINICAL SIGNIFICANCE : 

SPLENOMEGALY : CLINICAL SIGNIFICANCE Spleen size is not a reliable guide to spleen function.palpable spleen are not always abnormal,and hypersplenic spleens are not always palpable.Patients with emphysema and low diaphragms commonly have palpable but normal sized spleen.one study showed that 3 % of 2200 healthy ,college students had palpable spleens and another study showed that almost 5% of hospital patients with normal spleens by scan were found to have palpable spleens by their physicians.In contrast clinical splenomegaly is rarely noted in immune thrombocytopenic purpura , despite avid destruction of antibody-coated platelets by the spleen. Nevertheless, a palpable spleen must be considered a phystcal finding that demands further evaluation.

Signs and symptoms : 

Signs and symptoms Left upper quadrant pain[dragging pain] Fullness or early satiety Left upper quadrant pain sometimes radiating to left shoulder [splenic infarct] Anemia due to red cell destruction Palpable abdominal mass Disturbances of bowel habits Sometimes rectal or vaginal prolapse

METHODS FOR EVALUATION OF SPLENOMEGALY : 

METHODS FOR EVALUATION OF SPLENOMEGALY 1.PHYSICAL EXAMINATION:most practical & cost effective 2.Radiology: Ultrasonography Computed tomography Liver-spleen scanning Positron emission tomography MRI Nuclear medicine scans 3.Biopsy: Needle aspiration Splenectomy

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Note: USG is the current procedure of choice for routine assessment of spleen size,because it has high sensitivity & specificity & is safe , non invasive ,quick,mobile &less costly.Nuclear medicine scans are accurate,sensitive & reliable but are costly and require immobile equipment.CT &MRI provide accurate determination of spleen size , but the equipment is immobile & costly.MRI offers no advantage over CT. Changes in spleen structure such as mass lesions , infarcts , inhomogenous infiltrates and cysts are more readily assessed by CT , MRI .or USG. but none of these techniques is very reliable in the detection of patchy infiltration. Radionuclide scans such as gallium scans can identify active lymphoma or infections. Because of its propensity to bleed & its location , needle aspiration or cutting needle biopsy of the spleen is rarely performed.

Clinical methods of detecting splenomegaly : 

Clinical methods of detecting splenomegaly Spleen must be twice its normal size to be detected clinically.When enlarged it extends from left costal margin towards the right iliac fossa.It moves freely with respiration. Splenic swelling has a sharp anterior border where splenic notch can be felt.

Palpation: : 

Palpation: There are four methods of palpation of spleen: 1. The right hand of clinician is placed parallel to the left costal margin at the level of umbilicus and the patient is asked to breathe in and out.during expiration hand is gradually slided towards the left costal margin till the splenic swelling touches the lateral border of the index finger during inspiration 2. Clinician’s.left hand is placed over left lower ribs and the skin is slided downwards so that right hand gets an extra bit of skin to insinuate beneath the left costal margin.By this method even a relatively smaller spleen can be palpated.An enlarged spleen appears just below the tip of 10th rib.

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3. Another method is that when the right hand reaches the costal margin at the tip of the 10th rib clinician’s left hand is put around the lower left rib cage and it is pushed forward with each inspiration. This method occasionally lifts a slightly enlarged spleen forwards enough to make it palpable. 4. From above spleen may be conveniently palpated with two hands arching below the left costal margin while the patient is asked to take deep breaths in & out slowly.The hands are moved further downwards & laterally with each expiration waiting for the enlarged spleen to knock at the fingers during inspiration while the fingers are kept static.

Percussion: : 

Percussion: Three methods have been described….. 1.Nixon’s method: the patient is placed on the right side so that the spleen lies above the colon and stomach. Percussion begins at the level of pulmonary resonance in the posterior axillary line and proceeds diagonally along a perpendicular line toward the lower mid anterior costal margin.The upper border of dullness is normally 6 to 8 cm above the costal margin.Dullness >8 cm in an adult is presumed to indicate splenic enlargement.

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2.Castell’s method :It involves first placing the patient in the supine position. With the patient in full inspiration and then full expiration, percuss the area of the lowest intercostal space (eighth or ninth) in the left anterior axillary line. If the note changes from resonant on full expiration to dull on full inspiration, the sign is regarded as positive. The resonant note heard upon full expiration is likely to be due to the air-filled stomach or splenic flexure of the colon. When the patient inspires, the spleen moves inferiorly along the posterolateral abdominal wall. If the spleen is enlarged enough that the inferior pole reaches the eighth or ninth intercostal space, a dull percussion note will be appreciated, indicating splenomegaly

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3.Percussion of Traube’s space:borders of Traube’s space are 6th rib superiorly , left midaxillary line laterally & the left costal margin inferiorly.During normal breathing , this space is percussed from medial to lateral margins, yielding a normal resonant sound.A dull percussion note suggests spleenomegaly.

Splenomegaly grouped on the basis of the pathogenic mechanism : 

Splenomegaly grouped on the basis of the pathogenic mechanism Increased function : A) Removal of defective RBCs :SpherocytosisThalassemiaHemoglobinopathiesNutritional anemiasearly sickle cell anemia B)Immune hyperplasiaResponse to infection (viral,bacterial,fungal,parasitic)Mononucleosis, AIDS, viral hepatitissubacute bacterial endocarditis, bacterial septicemiasplenic abscess, typhoid feverbrucellosis, leptospirosis, tuberculosishistoplasmosismalaria, leishmaniasis, trypanosomiasisehrlichiosis

Splenomegaly grouped on the basis of the pathogenic mechanism : 

Splenomegaly grouped on the basis of the pathogenic mechanism Disordered immunoregulationRheumatoid arthritis,SLESerum sicknessAutoimmune hemolytic anemiaImmune thrombocytopeniasarcoidosis , drug reactions Extramedullary hematopoiesisMyelofibrosisMarrow infiltration by tumors, leukemiasmarrow damage by radiation, toxins

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Abnormal blood flow Organ Failure Cirrhosiscongestive heart failureVascularhepatic vein obstructionportal vein obstructionBudd-Chiari syndromesplenic vein obstructionInfectionshepatic schistosomiasishepatic echinococcosis

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Infiltration Metabolic diseasesGauchers diseaseNiemann-Pick diseaseHurler syndrome and other MucopolysaccharidosesAmyloidosisTangier diseaseBenign and malignant infiltrationsLeukemias(acute,chronic,lymphoid and myeloid)lymphomas(Hodgkins and non-hodgkins)myeloproliferative disordersmetastatic tumors(commonly melanoma)histiocytosis XHemangioma,lymphangiomasplenic cystshamartomaseosinophilic granuloma

ISOLATED SPLENOMEGALY : 

ISOLATED SPLENOMEGALY The common conditions that result in isolated splenomegaly are : 1. Haematological : 25-66 % 2. Hepatic : 12-46 % 3. Infective : 13-25 % 4. Primary splenic : 2-5 % 5. Others : 8-21 %

CLINICAL APPROACH : 

CLINICAL APPROACH The approach to splenomegaly is basically to find and manage the cause and seldom involves a splenectomy. The diagnosis of the cause requires a complete history and thorough physical examination with the following points of importance: • Recent trauma in area of spleen • Associated symptoms / asymptomatic (causes of initially asymptomatic splenomegaly may include early myelproliferaitve disorders, splenic cyst , mild hereditary spherocytosis and even cases of Gauchers disease and cirrhosis) • • Family history • Features of an acute / chronic infection • Lymphadenopathy • Size and consistency of the spleen

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Splenomegaly may represent a manifestation of primary disease or associated disease or probably due to the previous illness not recognised . the differential diagnosis of splenomegaly differs with splenic size at presentation, age of the patient , clinical features , hepatomegaly and lymphadenopathy. Haematological diseases had significant positive association with massive splenomegaly , lymphadenopathy , and blood cytosis, i.e., erythrocytosis , leucocytosis , thrombocytosis. Hepatic diseases had highly significant positive association with hepatomegaly , abnormal LFTs and blood cytopenia . infectious diseases showed a positive association with fever . left upper abdominal pain had significantly positive association with haematological and primary splenic diseases .

Differential Diagnosis : 

Differential Diagnosis 1.Hemolytic anaemia : cogenital : hereditary spherocytosis acquired Hereditary spherocytosis # autosomal dominant # incidence of 1: 1000 to 1:4500. # patient present with anemia , splenoimegaly and jaundice # jaundice may be intermittent # pigmented gall stones are common due to increased bile pigment production # haemolytic rate may increase transiently during systemic infections # a family history may be elicited # history of biliary colic may be present # sometimes severe crisis of red cell destruction may occur; RBC count may fall down to 2 millions during the attack .such attacks are vcharacterised by abdominal pain , nausea , vomiting and pyrexia besides usual extreme pallor and jaundice . these crisis may be precipitated by acute infection and may cost life . # on examination : spleen is always enlarged , liver may be palpable , pallor present , icterus present # lab findings : spherocytes seen on peripheral smear , MCV is normal or slightly decreased , MCHC is increased , osmotic fragility test will show increased fragility of the RBCs , reticulocyte count will give an index of severity of this condition ; after a crisis this will be greatly increased , fecal urobilinogen will be increased , use of radioactive chromium by labelling the patient’s own red cells and by daily scanning over the spleen will show the degree of red cell sequesteration by the spleen . if the sequesteration is high , splenectomy can be considered

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Acquired haemolytic anemia # autoantibodies are produced which attach themselves to the circulating RBC and this results in their early dissolution. #coombs test is usually but not always positive # patients are usually middle aged or elderly subjects # family history usually absent # splenomegaly present in 50% cases # liver may be palpable # treatment : mild hemolysis- NO treatment Clinically significant hemolysis : glucocorticoids , splenectomy (for patients who cannot tolerate or fail to respond to steroids) immunosuppressive drugs , blood transfusion.

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CML Causes, incidence, and risk factors    CML can occur in adults (usually middle-aged) and children. The disease affects 1 to 2 people per 100,000 and accounts for 7 - 20% cases of leukemia. It is usually associated with a chromosome abnormality called the Philadelphia chromosome. CML causes rapid growth of the blood-forming cells (myeloid precursors) in the bone marrow, peripheral blood, and body tissues. Exposure to ionizing radiation is one possible trigger for this chromosome abnormality. Such exposure could occur from a nuclear disaster or from treatment of a previous cancer, like thyroid cancer or Hodgkin's lymphoma. However, the vast majority of people treated for cancer with radiation do not go on to develop leukemia. It takes many years to develop leukemia from this cause. Symptoms    Chronic myelogenous leukemia is grouped into several phases. The chronic phase that can last for months or years. The disease may have few or no symptoms during this time. Most people are diagnosed by during this stage, when they are being tested for something else. The accelerated phase is a more dangerous phase, during which the leukemia cells grow more quickly. Acceleration of the disease may be associated with fever (without infection), bone pain, and a swollen spleen. If untreated, CML progresses to the blast crisis phase. This phase is very difficult to treat and is marked by a very high count of immature white blood cells (leukemia cells). Bleeding and infection may occur due to bone marrow failure. Other possible symptoms include: Fatigue Weakness Excessive sweating (night sweats) Low-grade fever Pressure under the left ribs from an swollen spleen Bleeding and bruising Sudden appearance of small red marks on the skin (petechiae)

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Signs and tests    A physical examination often reveals an enlarged spleen. A CBC shows an increased number of white blood cells. Other tests that may be done include: CBC differential Bone marrow aspiration Testing for the presence of the Philadelphia chromosome Molecular assay for the bcr-abl gene This disease may also alter the results of the following tests: Vitamin B-12 level Uric acid Platelet count Treatment    Imatinib (Gleevec) is the first line of therapy for all patients. Gleevec blocks the Philadelphia chromosome and is assosicated with very high rates of remission. Similar drugs are being developed. Sometimes a chemotherapy medicine called hydroxyurea (Hydrea) is used temporarily to control the white blood cell count. The only known cure for CMS is a bone marrow transplant or stem cell transplantation.

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Hairy cell leukemia Definition    Hairy cell leukemia (HCL) is a rare cancer of the blood. It affects B cells, a type of white blood cell (lymphocyte). Causes, incidence, and risk factors    HCL is caused by the abnormal growth of B cells. The cells can look "hairy" under the microscope because they have fine projections coming from their surface.and show a characteristic staining pattern with tartarate – resistant acid phosphatase The cause of this disease is unknown. It affects men more often than women. The average age of onset is 55. Symptoms    Weakness Fatigue Weight loss Easy bruising or bleeding Recurrent infections and fevers Excessive sweating (especially at night) Swollen lymph glands Feeling full after eating only a small amount (early satiety)

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Signs and tests   Splenomegaly, pallor   . An abdominal CT scan may be done. A complete blood count shows low levels of white and red blood cells, as well as platelets. Blood tests and a bone marrow biopsy can detect hairy cells Treatment    It is responsive to chemotherapy with interferon – alpha , pentostatin or cladribine . Removing the spleen may improve blood counts, but is unlikely to cure the disease. Antibiotics can be used to treat infections. People with low blood counts will receive growth factors and possible transfusions. Complications    The low blood counts caused by hairy cell leukemia can lead to infections, fatigue, and excessive bleeding.

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Splenic vein occlusion # isolated splenic vein thrombosis causes a rare syndrome characterised by left sided portal hypertension , upper GI bleeding , splenomegaly & occasionally hypersplenism #may present as upper GI bleeding in 45% cases #most of the cases due to primary pancreatic disease including acute& chronic pancreatitis & pancreatic tumors # splenomegaly found in 1/3rd cases # in this form of portal hypertension more than 90% patients are relieved of there GI bleeding by splenectomy . # definitive surgery for pancreatic disease may be required #embolisation of splenic artery is less satisfactory and may lead to splenic abscess

PORTAL HYPERTENSION : 

PORTAL HYPERTENSION Portal hypertension is caused most often by cirrhosis (in developed countries), schistosomiasis (in endemic areas), or hepatic vascular abnormalities. Consequences include esophageal varices and portal-systemic encephalopathy. Diagnosis is based on clinical criteria, often in conjunction with imaging studies and endoscopy. Treatment involves prevention of GI bleeding with endoscopy, drugs, or both, and sometimes with portocaval shunting. The portal vein, formed by the superior mesenteric and splenic veins, drains blood from the abdominal GI tract, spleen, and pancreas into the liver. Within reticuloendothelium-lined blood channels (sinusoids), blood from the terminal portal venules merges with hepatic arterial blood. Blood flows out of the sinusoids via the hepatic veins into the inferior vena cava. Normal portal pressure is 5 to 10 mm Hg (7 to 14 cm H2O), which exceeds inferior vena caval pressure by 4 to 5 mm Hg (portal venous gradient). Higher values are defined as portal hypertension.

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Portal hypertension is asymptomatic; symptoms and signs result from its complications. The most dangerous is acute variceal bleeding (see GI Bleeding: Varices). Patients typically present with sudden painless upper GI bleeding, often massive. Bleeding from portal hypertensive gastropathy is often subacute or chronic. Ascites, splenomegaly, or portal-systemic encephalopathy may be present. Portal hypertension is inferred in a patient with chronic liver disease by the presence of collateral circulation, splenomegaly, ascites, or portal-systemic encephalopathy. Proof requires direct portal pressure measurement by a transjugular catheter, which is invasive and usually not performed. Imaging may help when cirrhosis is suspected. Ultrasound or CT often reveals dilated intra-abdominal collaterals, and Doppler ultrasound can determine portal vein patency and flow. Esophagogastric varices and portal hypertensive gastropathy are best diagnosed by endoscopy, which may also identify predictors of esophagogastric variceal bleeding (eg, red markings on a varix).

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TREATMENT Usual treatment of acute variceal bleed such as banding & sclerotherapy Long-term drug therapy for varices that have bled involves β‑blockers Isosorbide mononitrate Transjugular Intrahepatic Portosystemic Shunt Reduces immediate deaths Long term benefit is not known Liver transplant Hypersplenism requires no specific treatment, and splenectomy should be avoided

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Felty syndrome Patients with rheumatoid arthritis may develop leukopenia : this is known as felty syndrome and it is associated with splenomegaly The cause of Felty syndrome is unknown. It is more common in people who have had rheumatoid arthritis for a long time. 5-10% of patients with RA have splenomegaly but felty syndrome occurs in 1% of all patients with RA Symptoms    General feeling of discomfort (malaise) Fatigue Loss of appetite Unintentional weight loss Pale-looking skin Joint swelling, stiffness, pain, and deformity Recurrent infections Eye burning or discharge Leg ulcer pigmentations

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Signs and tests    A physical examination shows splenomegaly and possibly hepatomegaly and lymph nodes. Joints may show signs of rheumatoid arthritis. A CBC (complete blood count) may show decreased number of white blood cells called neutrophils While pooling of leukocytes has been suggested as probable cause of leukopenia , the occurrence of IgG antibodies against neutrophils suggest more complex immunological theory . level of IgG antibodies return to normal; after splenectomy . anemia is common and may be due to plasma expansion Treatment    Persons with this syndrome are usually already receiving treatment for rheumatoid arthritis. Although splenectomy was done before but now there is no evidence that it alters the course of the disease but some patients do benefit from it. Expectations (prognosis)    Progression of rheumatoid arthritis is likely. Complications    Recurrent infections may develop

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Tropical splenomegaly (Hyper-reactive malarial splenomegaly; Big spleen disease) Tropical Splenomegaly Syndrome or Big Spleen Disease is massive enlargement of the spleen resulting from abnormal immune response to repeated attacks of malaria. It is seen among residents of endemic areas of malaria and it is not species specific. Tropical Splenomegaly Syndrome is characterised by massive splenomegaly, hepatomegaly, marked elevations in levels of serum Ig M and malaria antibody. Hepatic sinusoidal lymphocytosis is also seen. In about 10% of African patients, it may be associated with peripheral lymphocytosis (B cells). The interaction of repeated malarial infection and unknown host factors results in the production of cytotoxic Ig M antisuppressor lymphocyte (CD8+) antibodies. This causes inhibition of suppressor T cells, which normally regulate IgM production. This leads to uninhibited B cell production of IgM and the formation of cryoglobulins (IgM aggregates and immune complexes). The need to clear these macromolecular aggregates stimulates the reticuloendothelial system, resulting in hyperplasia. This causes the progressive and massive enlargement of the spleen and hepatomegaly.

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The spleen is massively enlarged. It shows dilated sinusoids lined with reticulum cells showing marked erythrophagocytosis and lymphocytic infiltration of the pulp. Liver exhibits sinusoidal dilatation, infiltration with lymphocytes and hyperplasia of the Kupffer's cells with phagocytosis of cellular debris and red cells. Most patients present during adult life. Patients present with dragging pain in the upper abdomen, or sometimes may even complain of a palpable mass. Some may experience recurrent sharp pains in the upper abdomen, probably due to perisplenitis or splenic infarcts. Some patients may have weight loss and cachexia. On examination, there is massive splenomegaly and hepatomegaly. The peripheral smear shows normocytic normochromic anemia with increased reticulocyte count. Leukopenia and thrombocytopenia may also be seen due to hypersplenism. Malarial parasites are not found in the peripheral blood. There is increase in the serum levels of polyclonal IgM with cryoglobulinemia, reduced C3 and the rheumatoid factor may be positive. The condition should be differentiated from other causes of splenomegaly in the tropics- Kala-Azar, Schistosomiasis, post-necrotic cirrhosis, thalassemia, leukemia, lymphoma, myelofibrosis, non-tropical idiopathic splenomegaly, Felty's syndrome etc. In patients with splenic lymphoma, more than 30% of circulating lymphocytes are villous and they can be differentiated from hairy-cell leukemia by their lack of CD25, CD11C and tartrate-resistant acid phosphate markers. Increased levels of IgM and antimalarial antibody, hepatic sinusoidal lymphocytosis on liver biopsy and response to antimalarial therapy (improvement in clinical condition as well as reduction in IgM and malarial antibody titre within three months of continuous antimalarial treatment) favour a diagnosis of tropical splenomegaly syndrome

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The disease generally runs a benign course. However, sometimes it may be associated with severe anemia, leading to congestive cardiac failure. These patients are also more prone for secondary bacterial infections of the skin and respiratory tract and have an increased mortality. Portal hypertension does not develop and the condition is reversible with antimalarial treatment. Some patients in Ghana were found to develop splenic lymphoma with hairy lymphocytes. The treatment of tropical splenomegaly syndrome involves administration of antimalarial prophylaxis for prolonged periods of time. This removes the antigenic stimulus provided by repeated malarial infection and allows the immune system to return to normal. The choice of antimalarial depends on the local sensitivity pattern. Chloroquine weekly or proguanil daily have been found to be useful. These drugs may have to be continued for long periods, possibly for life. Severe anemia may require blood transfusion. Splenectomy may do more harm than good and it may be beneficial in only patients with splenic lymphoma. Splenic irradiation or antimitotic therapy are not beneficial and may be even dangerous.

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Non tropical splenomegaly In non tropical countries , isolated splenomegaly is usually due to lymphoma , myeloid metaplasia or collagen vascular disease. Massive splenomegaly of unknown cause is also known as primary hypersplenism or Dacie’ syndrome . it is characterised by splenomegaly and pancytopenia which responds dramatically to splenectomy . Aetiology is not known . Histologically , the spleen reveals lymphoid hyperplasia with prominent germinal centres throughout the parenchyma

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Cyst Splenic cysts have been classified by Martin. Type I cysts are primary (true) cysts with a cellular lining, either parasitic or non-parasitic Non-parasitic type I cysts are either congenital or neoplastic. Type II cysts are secondary (false) cysts without cellular lining. Epidermoid cysts lined by squamous epithelium without skin adnexa and grossly a glistening inner surface are congenital cysts thought to be due to an embryonic defect in the aggregation of the mesodermal islands. Patients with congenital splenic cysts are younger than those with false cysts and marked female preponderance is observed. The majority of patients with splenic cysts have minor non-specific symptoms and the diagnosis is easily made by non-invasive imaging. Cysts arising from adjacent organs especially the tail of the pancreas can be an ultrasonic differential diagnosis. Aspiration of the cyst content will not further differentiate true from false cysts or subclassify the congenital Cysts

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Surgery is primarily recommended for the prevention or treatment of complications. Infection haemorrahage and rupture have been reported and may be life threateningThe percutaneous aspiration represents the final logical progression in favour of conservative approach. Since the cysts have an epithelial lining, the reaccumulation of fluid ultimately required partial spienectomy. In spite of partial splenectomy as the preferred approach, Brown et al reported that a total splenectomy was required in one out of seven patients, where the cyst was large, encompassing hilum with only tiny island of splenic tissueThis particular report includes a similar case where partial splenectomy was not feasible.The world literature reveals that infected lesions are treated by splenectomy or by incision and drainage. The first report of partial splenectomy for infected cyst is by Khan et alfrom Canada. The multiple adhesions all around and bacterial culture suggested infection of the cyst which necessitated total splenectomy. The haemorrhage in the cyst could be due to trauma, infection or spontaneous.Hydatid disease is the commonest cause of splenic cyst in this country. The infected hydatid cyst with adhesions all around is difficult to differentiate from infected congenital cyst

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Splenic abscess Splenic abscess is a rare entity, with a reported frequency of 0.14-0.7% in autopsy series. Its reported mortality rate is still high, up to 47%, and can potentially reach 100% among patients who do not receive antibiotic treatment. Appropriate management can decrease the mortality to 14%.The timely and widespread use of imaging methods (eg, CT scanning, ultrasound) facilitates early diagnosis and guides treatment, thus improving the prognosis. male predominance. All age groups affected, most typically after the fourth decade.

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Clinical a high index of suspicion, particularly in the higher risk clinical scenarios The classical triad of fever, left upper quadrant pain, and splenomegaly is seen in only about one third of patients.Fever (>90%) can be moderate, continuous, intermittent, or even absent. Abdominal pain (>60%) typically occurs suddenly, maximum in the left hypochondrium (>39%). Involvement of the diaphragmatic pleura can cause shoulder pain. The associated eponym is the Kehr sign,. Pleuritic chest pain around the left lung base (>15%) is aggravated by coughing or forced expiration.

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Physical examination  Abdominal tenderness (>50%)  There may be edema of the soft tissues overlying the spleen. Costovertebral tenderness may also be noted. Splenomegaly (<50%). Chest findings are nonspecific and reportedly include dullness at the left lung base (>30%), left basilar rales (>21%), or elevation of the left hemidiaphragm (>15%). Treatment Empiric broad -spectrum antibiotics have a primary role in the initial management of splenic abscesses. Percutaneous drainage has gained acceptance as an effective and less invasive treatment method than surgical intervention in selected patients.  The reported success rate is 67-100%. Such drainage preserves the spleen and avoids the risk of overwhelming postsplenectomy infections. Percutaneous drainage can also be used as a bridge to elective surgery in patients who are clinically unstable or in patients who have multiple comorbidities.Multilocular abscesses, ill-defined cavities, septations, and necrotic debris typically do not respond to percutaneous drainage. Surgery

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Lab Studies leukocytosis (WBC count, >20,000/mm3) with a left shift in most patients.. Recurrent positive blood cultures. Imaging Studies Abnormal chest radiograph findings in most patients Elevated left hemidiaphragm (>30%) Pleural effusion (>20%) Plain x-ray films of the abdomen are notoriously nonspecific in patients with a splenic abscess.  Findings on abdominal x-ray films can include abnormal soft tissue density or a gas collection in the left upper quadrant. Ultrasound is cost-effective, noninvasive, and readily available at the bedside around the clock. However, the evaluation is nonspecific and operator dependent CT scan is presently the criterion standard in helping to establish the diagnosis of splenic abscess. Reported sensitivity of the CT scan for this purpose typically approaches 100%. The characteristic image of splenic abscess reveals low-density lesions that fail to enhance after intravenous contrast. CT scan best delineates the size, topography, and access routes to the spleen and surrounding structures. CT-guided drainage can be performed during the examination.

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Medical therapy Early supportive care and parenteral broad-spectrum antibiotics are of paramount importance while further diagnostic and therapeutic arrangements are made. Besides the more common organisms isolated from splenic abscesses, mycobacteria, Candida, and Aspergillus should also be considered; these organisms account for 8% of the splenic abscesses in patients who are immunocompromised. Fungal abscesses are known to respond more favorably to antifungal treatment because they result more often from a disseminated infection. The mortality rate with antifungal therapy alone is 6%. Surgical therapy The invasive treatment of splenic abscess includes 3 options: percutaneous drainage, open or laparoscopic surgery (splenectomy), and open drainage.

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Percutaneous drainage indicated for easily accessible uniloculated or biloculated abscesses features, and also for very high-risk surgical patients who cannot tolerate general anesthesia or surgery. The procedure includes a risk of iatrogenic injury of the spleen, colon (splenic flexure), stomach, left kidney, and diaphragm.Other iatrogenic complications resulting from percutaneous drainage include hemorrhage, pleural empyema, pneumothorax, and enteric fistula. Splenectomy Splenectomy has long been considered the standard treatment of splenic abscess. Depending on the patient population, open splenectomy has a mortality rate of 0-17% and a morbidity rate of 28-43%. Open drainageOpen drainage is used in case of an inability to drain the abscess percutaneously. Depending on the location of the abscess, 1 of 3 access routes can be used: Transpleural Abdominal extraperitoneal Retroperitoneal

Hypersplenism : 

Hypersplenism This is a complication of splenomegaly & not a diagnosis It is characterised by splenomegaly , any combination of anemia , leucopenia or thrombocytopenia , compensatory bone marrow hyperplasia and improvement after splenectomy Aetiology of hypersplenism in different conditions : Disease mechanism a) hairy cell leukaemia retention of hairy cells in red pulp b) cirrhosis ,splenic vein thrombosis increased pooling of blood cells c) Felty syndrome immune system work hypertroph d)thalassaemia major RE system work hypertrophy

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Asymptomatic splenomegaly Patients with asymptomatic or incidently discovered splenomegaly present a difficult diagnostic problem . careful and periodic regular follow up of these patients is required to rule out serious causes such as malignancy .Causes of asymptomatic splenomegaly are : a) liver disease with portal hypertension b) splenic vein thrombosis c) agnogenic myeloid metaplasia d) Gaucher disease e) splenic cyst f) sarcoidosis g) amyloidosis h) mild hereditary spherocytosis i) early stages of polycythemia vera Malignancy was the most common cause of unexplained splenomegaly though benign neoplasms and reactive disorders were documented in 25% cases in a study .In another study a definitive histological diagnosis was established in 9 out of 10 diagnostic splenectomies , 7 of whom had lymphoma .In patients who are unwell or in which the appropriate investigations have not yielded a diagnosis , splenectomy may be considered

Essential investigations : 

Essential investigations RADIOLOGY CT , MRI. CHEST X RAY PROCEDURES Liver biopsy Bone marrow aspirate Bone marrow biopsy Lymph node biopsy Broncho alveolar lavage Upper g I endoscopy BLOOD RF ANF Coombs test Blood cultures Serology for HIV , hepatitis etc..

Splenectomy : 

Splenectomy INDICATIONS trauma – accidental , operative oncological – part of en bloc resection , diagnostic , therapeutic haematological – spherocytosis , ITP , hypersplenism portal hypertension – variceal surgery Indication is diagnostic in 10% of patients , therapeutic in 44% , staging for Hodgkin disease in20% , and incidental to another procedure in 26% CONTRAINDICATION bone marrow failure

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COMPLICATIONS hemorrhage from a slipped ligature from the splenic artery left basal atelectasis pleural effusion hematemesis from gastric mucosal damage and gastric dilatation Pancreatitis Pancreatic fistula Pancreatic abscess Thrombocytosis Septicemia from capsulated organism Post splenectomy septicaemia may result from Streptococcus pneumoniae , Nisseria meningitides , Haemophilus influenzae and E.coli.The risk is greater in the young patient , in splenectomised patients treated with chemoradiotherapy and in patients who have undergone splenectomy for thalassemia , sickle cell disease and auto immune anemia or thrombocytopenia .

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Susceptibility to infections by encapsulated organisms is more serious complication. Overall risk of sepsis in splenectomised patients is 7% in 10 yrs. Patient under 20 yrs of age are more susceptible to sepsis with S.pneumoniae Case fatality rate for pneumococcal sepsis is 50 – 80 % in such patients . About 15% of infections are poly microbial. Lung , skin & blood are the most common sites . No increase risk of viral infections. Susceptibility to bacterial infections relates to the inability to remove opsonised bacteria from the blood stream and a defect in making antibodies to T-cell independent antigens such as the polysaccharide components of bacterial capsules.

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Vaccines to be given : a)Pneumococcal in patients aged over 2 yrs (23-valent polysaccharide vaccine) b)Meningococcal vaccine Protection following vaccination is not always granted . Splenectomized patients are also susceptible to Babesiosis

EFFECT OF SPLENECTOMY ON HEMATOLOGICAL PROFILE : 

EFFECT OF SPLENECTOMY ON HEMATOLOGICAL PROFILE Immediate post- splenectomy period leukocytosis – upto 25000/ul thrombocytosis – upto 1000000/ul within 2 to 3 weeks blood cell counts & survival are usually normal chronic manifestations : anisocytosis , poikilocytosis , Howell – jolly bodies , Heinz bodies , basophilic stippling & nucleated erythrocytes.

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