SPLENOMEGALY- INTEGRATED

APPROACH TO DIAGNOSIS
GUIDE : DR. ATUL SHENDE CANDIDATE: DR. SARATH MENON.R DIVISION OF GASTROENTEROLOGY MGM MEDICAL COLLEGE,INDORE

INTRODUCTION

Definition & symptoms Classification Hypersplenism Etiology Step-wise approach

Associated features
Investigations

black

Galen source of black bile or melancholia

NORMAL SPLEEN

Normal size 12 cm length , 7 cm width (radionuclide scan) -13cm craniocaudal diamtr (USG) weight- < 250gm Located along- 9th, 10th,11th ribs mid-axillary Spleen should be twice the size to be PALPABLE

Palpable spleens are not always ABNORMAL 3% normal population has palpable spleen

FUNCTIONS

Quality control over RBC culling & pitting Synthesis of antibodies

Removal of antibody coated bacteria & RBC

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

TRAUBES SPACE

BIMANUAL PALPATION

DIFFERENCES
spleen kidney

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

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)

HYPERSLENISM

Splenomegaly Pancytopenia

Presence of hypercellular marrow

Reversal with splenectomy

SYMPTOMS OF SPLENOMEGALY

Pain Early satiety

Feeling of heaviness in LUQ

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

CAUSES OF SPLENOMEGALY

Infective Hyperplastic

Congestive
Infiltration

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

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

HYPERPLASTIC

Extramedullary hemopoeisis- myeloprolif.d/s - marrow damage - marrow infiltration

Reticulo endothelial hyperplasia (abn.RBC) - sickle cell d/s,spherocytosis,Hbnopathies, thalassemia major,PNH

INFILTRATIVE Malignant infiltration- CML,lymphoblastic - lymhomas, MPD, - angiosarcoma,tumors - metastasis (melanoma) benign - storage d/s Gauchers,Neiman-pick - amyloidosis - hurlers syndrome,MPS - cysts,fibromas,hemangiomas,hamartomas -Eosnophilic granulomas

DISORDERED IMMUNOREGULATION

Feltys syndrome- RA+ splenomegaly+leucopenia Systemic lupus erythromatosis

Collagen vascular diseases

Sarcoidosis Immune thrombocytopenia

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

MODERATE SPLENOMEGALY(4-8CM)
Cirrhosis Lymphomas Amyloid Splenic abscess,infarct Hemolytic anemias IMN

MILD SPLENOMEGALY (1-3CM)

Acute infective conditons Acute malaria,tyhoid,kala-azar,septicemias

STEP-WISE APPROACH TO SPLENOMEGALY

History Physical examination Laboratory testings Imaging Specialised testing

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

HISTORY

..CONT

Jaundice High risk sexual behavior (AIDS) Past medical history Drugs

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

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

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

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

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

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- feltys syndrome,septicemia

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

SUMMARY

Splenomegaly major physical finding Step wise approach- history,physical exam

Look for associated features

Lab investigation & Imaging Search for etiology & treat