Approach to splenomegaly

SPLENOMEGALY- INTEGRATED APPROACH TO DIAGNOSISCANDIDATE: DR. SARATH MENON.RDIVISION OF GASTROENTEROLOGYMGM MEDICAL COLLEGE,INDORE

INTRODUCTION Definition & symptoms

Classification

Hypersplenism

Etiology

Step-wise approach

Associated features

Investigations

Galen –source of black “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

TRAUBE’S SPACE

BIMANUAL PALPATION

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

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 –Gaucher’s,Neiman-pick - amyloidosis - hurler’s syndrome,MPS -

cysts,fibromas,hemangiomas,hamartomas -Eosnophilic granulomas

DISORDERED IMMUNOREGULATION Felty’s 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- felty’s 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