Approach to splenomegaly

INTEGRATED APPROACH TO SPLENOMEGALY

1. Splenomegaly - integrated approach to diagnosis CANDIDATE : DR. SARATH MENON.R DIVISION OF GASTROENTEROLOGY MGM MEDICAL COLLEGE,INDORE

2. introduction Definition & symptoms Classification Hypersplenism Etiology Step-wise approach Associated features Investigations

3. Galen –source of black “black bile’ or “melancholia”

4. Normal spleen Normal size – 12 cm length , 7 cm width (radionuclide scan) -13cm craniocaudal diamtr (USG) weight- < 250gm Located along- 9 th , 10 th ,11 th ribs mid- axillary Spleen should be twice the size to be PALPABLE Palpable spleens are not always ABNORMAL 3% normal population has palpable spleen

5. functions Quality control over RBC – culling & pitting Synthesis of antibodies Removal of antibody coated bacteria & RBC

6. Examination of spleen Inspection Percussion – nixon method - rt.lateral ducubitus , > 8cm - castells method supine,lower ICS ,full exp & insp splenomegaly =dullness - traubes sign supine,6 th rib,costal margin, anterior axill . line splenomegaly = dullness Palpation - bimanual method,hooking maneuver

7. Traube’s space

8. Bimanual palpation

9. differences Sharp edge Notch –med border Cross midline Moves with respiration Cannot get above it Round edge No notch Not cross midline Not moves with resp. Can get above it spleen kidney

10. splenomegaly Mild,moderate,massive Massive - beyond umblicus , crosses mid line into pelvis (>8cm) Moderate - b/w costal margin & umblicus (4-8cm) Mild - just palpable (1-3cm)

11. hyperslenism Splenomegaly Pancytopenia Presence of hypercellular marrow Reversal with splenectomy

12. Symptoms of splenomegaly Pain Early satiety Feeling of heaviness in LUQ

13. Mechanism of spenomegaly Reactive Reticulo -endothelial hyperplasia Lymphoid hyperplasia Proliferation of lymphoma cells Infiltration by abnormal cells Extramedullary hemopoeisis Proliferation of macrophages d/t RBC destruction Vascular congestion

14. Causes of splenomegaly Infective Hyperplastic Congestive Infiltration

15. infective Acute & subacute - IMN, infective endocarditis , severe pyogenic inf. Viral hepatitis,CMV,AIDS Chronic - TB,syphilis,brucellosis Tropical splenomegaly Malaria,kala azar , trypanosomiasis

16. congestive Intra hepatic obst.portal hypertension - cirrhosis,biliary cirrhosis,hemochromatosis - primary sclerosing cholangitis Extra-hepatic portal hypertension - venous malf,thrombosis,stenosis - ext.occlusion of portal,splenic vein Chronic passive congestion of cardiac origin

17. hyperplastic Extramedullary hemopoeisis - myeloprolif.d /s - marrow damage - marrow infiltration Reticulo endothelial hyperplasia –(abn.RBC) - sickle cell d/ s,spherocytosis,Hbnopathies , thalassemia major,PNH

18. infiltrative Malignant infiltration- CML,lymphoblastic - lymhomas , MPD, - angiosarcoma,tumors - metastasis (melanoma) benign - - storage d/s – Gaucher’s,Neiman -pick - amyloidosis - hurler’s syndrome,MPS - cysts,fibromas,hemangiomas,hamartomas - Eosnophilic granulomas

19. Disordered immunoregulation Felty’s syndrome- RA+ splenomegaly+leucopenia Systemic lupus erythromatosis Collagen vascular diseases Sarcoidosis Immune thrombocytopenia

20. Massive splenomegaly (>8cm >1000gm) Myeloproliferative disorder Chronic malaria,kala-azar (trop. Splenomegaly ) Storage disorders Thalassemia major Sarcoidosis Hairy cell leukemia Gaucher disease Diffuse splenic hemangiomatosis

21. Moderate splenomegaly (4-8cm) Cirrhosis Lymphomas‘ Amyloid Splenic abscess,infarct Hemolytic anemias IMN

22. Mild splenomegaly (1-3cm) Acute infective conditons Acute malaria,tyhoid,kala-azar,septicemias

23. Step-wise approach to splenomegaly History Physical examination Laboratory testings Imaging Specialised testing

24. history Age ,gender Race h/o recent infections like malaria Fever,weight loss,sweating ( lymphomas,infections ) Pruritis Abnormal bleeding/bruising Joint pain h/o alcholism h/o trauma h/o neonatal umblical sepsis Residence & travel abroad

25. History …..cont Jaundice High risk sexual behavior (AIDS) Past medical history Drugs

26. Physical examination Size of the spleen Hepatomegaly Lymphadenopathy Fever Icterus Bruising,petechiae Oral & supf.sepsis Stigmata of liver disease Stigmata of RA/SLE Splinter hemorrhage,retinal hemorrhage Cardiac murmurs

27. Lab investigations CBC Blood smear Retic count Blood C/S Serology ( fungal,viral,parasitic ) LFT Hb electropheresis / coombs test Coag.profile Amylase/lipase AMA, Anti CCP,RA factor Bone marrow analysis

28. Imaging USG- sensitive & specific non-invasive CT scan – etiology of splenomegaly - liver size,heterogenecity - splenic mets , abscess,calcf.,cysts - retro peritoneal LN - craniocaudal ln > 10 cm Liver- spleen colloid scan- (RBC –Cr51,Tc99) - hepatic steatosis,SOL,splenic functions - PHT,colloid shift + MRI/ Doppler usg - portal/ splenic vein thrombosis - cavernomas

29. imaging MRI scan- liver hemangiomas hemochromatosis erlenmeyer flask sign( Gaucher ) PET scan - Dx & staging of lymphomas - determine metabolic cells in spleen

30. Specialised testing Abd.fat pad aspiration JAK-2 mutation Gene testing( bcr-abl ,C282Y) Enzyme testing Lymph node biopsy FNAB spleen Splenectomy Lung or skin biopsy Liver biopsy

31. Special situations associated with splenomegaly Fever- typhoid,malaria,kalaazar , infect.endocarditis , leukemia,lymphoma Tender spleen- rupture,abscess,infarct a/c illness+ anemia- AIHA,leukemia Fever + LN- IMN,leukemia,lymhomas,SLE,sarcoid Anemia- hemolytic anemia,hemoglobinopathies Jaundice – cirrhosis,hemolytic anemia Pulsatile spleen- aneurysm High ESR- connective tissue disorder Leukopenia - felty’s syndrome,septicemia

32. Tropical splenomegaly ( hms ) Massive splenomegaly Endemic areas of malaria,kala-azar IgM antibodies + No parasite in blood Lymhocytic infiltration of splenic sinusoids Long term anti- malarials

33. summary Splenomegaly – major physical finding Step wise approach- history,physical exam Look for associated features Lab investigation & Imaging Search for etiology & treat