logging in or signing up APPROACH TO SPLENOMEGALY baiju.faizal Download Post to : URL : Related Presentations : Share Add to Flag Embed Email Send to Blogs and Networks Add to Channel Uploaded from authorPOINT lite Insert YouTube videos in PowerPont slides with aS Desktop Copy embed code: Embed: Flash iPad Dynamic Copy Does not support media & animations Automatically changes to Flash or non-Flash embed WordPress Embed Customize Embed URL: Copy Thumbnail: Copy The presentation is successfully added In Your Favorites. Views: 1675 Category: Entertainment License: All Rights Reserved Like it (0) Dislike it (0) Added: April 02, 2012 This Presentation is Public Favorites: 0 Presentation Description No description available. Comments Posting comment... Premium member Presentation Transcript SPLENOMEGALY -an approach: SPLENOMEGALY -an approach Dr. B. P. Faizal , Dr. RojithFUNCTIONS OF SPLEEN: FUNCTIONS OF SPLEEN Clears microorganisms from blood Synthesis of immunoglobulins Destruction of abnormal RBCs Embryonic and extramedullary hematopoiesisWHEN 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 diseaseETIOLOGY 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 NigeriaCLASSIFICATION 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 spleenCLASSIFICATION OF SPLENOMEGALY: CLASSIFICATION OF SPLENOMEGALY Mild splenomegaly - spleen <5 cm below left costal margin Moderate splenomegaly – 5-8 cm Massive splenomegaly >8 cmDISTINGUISHING 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- ThallasaemiaSYMPTOMS : 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, HCLCLINICAL SIGNS: CLINICAL SIGNS Plethora , hypertension , retinal vein distension Gynecomastia , clubbing, spider naevi , other signs of liver cell failure - CirrhosisCLINICAL FINDINGS: CLINICAL FINDINGS SPLEEN WITH LYMPH NODES CLL Mantle cell lymphoma HEPATOSPLENOMEGALY Kala azar Myelofibrosis , HCL Post sinusoidal PHT MASSIVE SPLEEN ALONE HMS CML NCPFINVESTIGATIONS: 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 splenectomyNCPF: 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 stigmataNCPF: 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 2002SCHISTOSOMIASIS: SCHISTOSOMIASIS S.Mansoni , S.Hematobium Hepatosplenomegaly with portal hypertension Few reports from India from parts of Maharashtra and AssamTSS: TSSCRITERIA 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 1991NonTropical 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 98CRITERIA FOR HYPERSPLENISM: CRITERIA FOR HYPERSPLENISM Splenomegaly Cytopenia Normal or hyperplastic marrow Response to splenectomyCONCLUSIONS: 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-1996THANK YOU: THANK YOU You do not have the permission to view this presentation. In order to view it, please contact the author of the presentation.
APPROACH TO SPLENOMEGALY baiju.faizal Download Post to : URL : Related Presentations : Share Add to Flag Embed Email Send to Blogs and Networks Add to Channel Uploaded from authorPOINT lite Insert YouTube videos in PowerPont slides with aS Desktop Copy embed code: Embed: Flash iPad Dynamic Copy Does not support media & animations Automatically changes to Flash or non-Flash embed WordPress Embed Customize Embed URL: Copy Thumbnail: Copy The presentation is successfully added In Your Favorites. Views: 1675 Category: Entertainment License: All Rights Reserved Like it (0) Dislike it (0) Added: April 02, 2012 This Presentation is Public Favorites: 0 Presentation Description No description available. Comments Posting comment... Premium member Presentation Transcript SPLENOMEGALY -an approach: SPLENOMEGALY -an approach Dr. B. P. Faizal , Dr. RojithFUNCTIONS OF SPLEEN: FUNCTIONS OF SPLEEN Clears microorganisms from blood Synthesis of immunoglobulins Destruction of abnormal RBCs Embryonic and extramedullary hematopoiesisWHEN 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 diseaseETIOLOGY 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 NigeriaCLASSIFICATION 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 spleenCLASSIFICATION OF SPLENOMEGALY: CLASSIFICATION OF SPLENOMEGALY Mild splenomegaly - spleen <5 cm below left costal margin Moderate splenomegaly – 5-8 cm Massive splenomegaly >8 cmDISTINGUISHING 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- ThallasaemiaSYMPTOMS : 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, HCLCLINICAL SIGNS: CLINICAL SIGNS Plethora , hypertension , retinal vein distension Gynecomastia , clubbing, spider naevi , other signs of liver cell failure - CirrhosisCLINICAL FINDINGS: CLINICAL FINDINGS SPLEEN WITH LYMPH NODES CLL Mantle cell lymphoma HEPATOSPLENOMEGALY Kala azar Myelofibrosis , HCL Post sinusoidal PHT MASSIVE SPLEEN ALONE HMS CML NCPFINVESTIGATIONS: 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 splenectomyNCPF: 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 stigmataNCPF: 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 2002SCHISTOSOMIASIS: SCHISTOSOMIASIS S.Mansoni , S.Hematobium Hepatosplenomegaly with portal hypertension Few reports from India from parts of Maharashtra and AssamTSS: TSSCRITERIA 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 1991NonTropical 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 98CRITERIA FOR HYPERSPLENISM: CRITERIA FOR HYPERSPLENISM Splenomegaly Cytopenia Normal or hyperplastic marrow Response to splenectomyCONCLUSIONS: 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-1996THANK YOU: THANK YOU