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