APPROACH TO SPLENOMEGALY

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SPLENOMEGALY -an approach:

SPLENOMEGALY -an approach Dr. B. P. Faizal , Dr. Rojith

FUNCTIONS OF SPLEEN:

FUNCTIONS OF SPLEEN Clears microorganisms from blood Synthesis of immunoglobulins Destruction of abnormal RBCs Embryonic and extramedullary hematopoiesis

WHEN DOES SPLEEN ENLARGE?:

WHEN DOES SPLEEN ENLARGE? Increased function A.Removal of defective RBC B.Immune hyperplasia C. Extramedullary hematopoeisis Abnormal bld flow Infiltration

Differentials..:

Differentials.. Portal hypertension - Presinusoidal , sinusoidal or post sinusoidal Hyperreactive malarial splenomegaly Hematological diseases – CML , Myelofibrosis , MCL , SMCL , CLL ,PV, Hairy cell leukemia, beta thallasaemia Infections – Kala azar , Schistosomiasis Infiltrative disorders – Gaucher’s disease

ETIOLOGY BASED ON LOCATION:

ETIOLOGY BASED ON LOCATION WESTERN WORLD THIRD WORLD Hemat – 25 -66% Portal Htn /hepatic – 12-45% Infective -13 -25% Primary splenic – 2-5% Undiagnosed- 8-21% Acta hematol 1999- 317 pts HMS –30 - 41% Hemat – 10- 30% Infective – 15 -20% Undiagnosed – 15 -25 % Lancet 2002-221 pts- Ghana and Nigeria

CLASSIFICATION OF SPLENOMEGALY- HACKETT:

CLASSIFICATION OF SPLENOMEGALY- HACKETT 0 – Spleen not palpable 1 – spleen just palpable below LCM, on deep inspiration 2 – spleen palpable< halfway between CM and umblicus 3 – spleen palpable> halfway to umblicus but not beyond it 4 – Spleen palpable below umblicus but not below horizontal line midway between umblicus and pubic symphysis 5 – lower than 4 1,2- mild spleen 3- moderate spleen 4,5 – massive spleen

CLASSIFICATION OF SPLENOMEGALY:

CLASSIFICATION OF SPLENOMEGALY Mild splenomegaly - spleen <5 cm below left costal margin Moderate splenomegaly – 5-8 cm Massive splenomegaly >8 cm

DISTINGUISHING SYMPTOMS:

DISTINGUISHING SYMPTOMS Age - < 20 years -EHPVO , Gaucher’s 10- 30 years – Kala azar , HMS 25 -40 years – NCPF, CML 60 – 70 years – CLL, HCL, PV, MCL, SMCL, Myelofibrosis Sex - F> M - NCPF(1.7: 1), HMS, CLL M> F - Kala azar (5.5:1), HCL(4:1)

DISTINGUISHING FEATURES :

DISTINGUISHING FEATURES Location – Kala azar – 90% -Bihar, West Bengal, UP, Bangladesh Specific subsects – Gaucher’s - Ashkenazi Jews Family history- Thallasaemia

SYMPTOMS :

SYMPTOMS Hematemesis – 1 st presenting symptom in NCPF, also occurs in cirrhosis Fever – Kala azar , myelofibrosis , ? HMS , SMZL Fatigue, abdominal pain, fullness, bleed Headache, dizziness, pruritis - PV Asymptomatic – CLL, HCL

CLINICAL SIGNS:

CLINICAL SIGNS Plethora , hypertension , retinal vein distension Gynecomastia , clubbing, spider naevi , other signs of liver cell failure - Cirrhosis

CLINICAL FINDINGS:

CLINICAL FINDINGS SPLEEN WITH LYMPH NODES CLL Mantle cell lymphoma HEPATOSPLENOMEGALY Kala azar Myelofibrosis , HCL Post sinusoidal PHT MASSIVE SPLEEN ALONE HMS CML NCPF

INVESTIGATIONS:

INVESTIGATIONS Blood counts Peripheral smear Liver function test and LDH Ultrasound abdomen Specific investigations - Hb electrophoresis - Viral markers - UGI scopy , Doppler of portal system - Antimalarial antibodies - splenic aspirate for LD bodies - Biopsy – Lymph node , liver, bone marrow Diagnostic splenectomy

NCPF:

NCPF Idiopathic portal hypertension – Japan Etiology – 1.bacterial infection from gut with repeated septic embolisation of portal circulation 2. Auto immune 3. Arsenic 3 rd - 4 th decade, F:M 1.7:1 , 15% of portal Htn 2/3 have massive splenomegaly Presenting symptom - hematemesis No cirrhosis/ encephalopathy/ CLD stigmata

NCPF:

NCPF Venography –no EHPVO, dilated portal and splenic v, intrahepatic portal v branches show abrupt narrowing at 2 nd and 3 rd order level Investigations- anaemia , hypersplenism 46%, U/S – liver normal/nodular Liver biopsy – portal fibrosis, tract edema, portal v sclerosis, subcapsular scarring Good prognosis if UGI bleed taken care of Dhiman et all- J Gastro & Hepat 2002

SCHISTOSOMIASIS:

SCHISTOSOMIASIS S.Mansoni , S.Hematobium Hepatosplenomegaly with portal hypertension Few reports from India from parts of Maharashtra and Assam

TSS:

TSS

CRITERIA FOR HMS:

CRITERIA FOR HMS Chronic massive splenomegaly ( Hackett 4/5) Increased IgM > 2SD above mean(>1000IU/ml) High titre of antimalarial antibodies Good response to prolonged antimalarial therapy(regression of spleen, raise in cell counts, decrease in IgM ) Abd swelling/dragging, 10-30yrs, F>M Susceptible to skin and respiratory infections Ind J Paeds 1991

NonTropical Splenomegaly Syndrome:

NonTropical Splenomegaly Syndrome Dacie’s syndrome Massive splenomegaly Primary hypersplenism Western countries Etiology uncertain 20% progress to lymphoma on follow up Br J Haemat 98

CRITERIA FOR HYPERSPLENISM:

CRITERIA FOR HYPERSPLENISM Splenomegaly Cytopenia Normal or hyperplastic marrow Response to splenectomy

CONCLUSIONS:

CONCLUSIONS Background history, presenting symptoms Careful evaluation Adenopathy , cytosis - HEMAT Abnormal LFT, cytopenia – HEPAT 15- 20%- undiagnosed- bone marrow, node,liver biopsy gives definite pointers Diagnostic /therapeutic splenectomy if all investigations inconclusive R Reilly at all – Am J Med-1996

THANK YOU:

THANK YOU

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