1 year male child b/b relatives with c/o lump in abdomen (lt. Hypochondriac and umbilical). Baby have h/o fever on and off 15 days not a/w chills. O/E normal. Afebrile x 2 days. Accepting feeds, active. No other complains. Kindly discuss differentials. Blood reports awaited.



D / D :- In this case : 1 ) Leukemia 2 ) Lymphomas 3 ) Thalassemias 4 ) Congenital Spherocytosis 5 ) Portal hypertension 6 ) Storage disorders 7 ) Kalaazar 8 ) Malaria 9 ) Malignancy 10) Hemolytic anemia 11) Cirrhosis with portal hypertension 12 ) ICC 13 ) Recurrent Jaundice - Liver disease 14 ) Cardiac causes e.g. CCF ( 1 ) Approach to Hepatosplenomegaly :- Inflammatory Reticuloendothelial Venous Storage Space Occupying Fat Metabolic Hyperplasia Congestion disorders lesion infiltration . Infection Septicaemia Cardiac failure Glycogen storage Abscess Malnutrition Cystic Fibrosis -congenital Malignancy Pericardial Galactossaemia Primary Neoplasm TPN Wilsons disease -viral hepatitis TB effusion Mucopolysaccharidosis Secondary Neoplasm Uncontrolled -parasites Constrictive Lipidosis diabetes mellitus -fungal pericarditis Tyrosinaemia Hepatotoxic drugs Autoimmune Budd Chiari Reyes Syndrome Toxins/Drugs IVC valves Biliary Tract Obstruction ( 2 ) APPROACH TO A CHILD WITH HEPATOSPLENOMEGALY :- Summary of pathophysiological mechanisms :- 1. Inflammation (jaundice is often present) 2. Kupffer cell hyperplasia with TB or other generalized infections 3. Congestions exclude cardiac causes 4. Storage products usually abnormal appearance, neurological signs and marked firm enlargement of liver and spleen. 5. Space occupying lesions 6. Fat Infiltration typical of Kwashiokor and malnutrition 7. Metabolic Disorders

Here the discussion should go for- Infant with Spleno-hepatomegaly (S>H)- probably of infective etiology and/ infiltrative or neoplastic. Common causes of Speno hepatomegaly are 1.Malaria 2.Sepsis 3.Typhod fever 4.Toxoplasmosis 5.Brucellosis 6. IE 7.Splenic infarction. Other causes- Viral hepatitis, Miliary TB , Hemolytic anemias , Lymphoma, Leukaemia and Gauchers disease Advice pt to undergo cocktail of Investigations one by one to rule out the above causes- Infective> Childhood neoplastic > H anemias > Storage/ infiltrative.

I agree

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By description and usg report it seems massive splenomegaly with mild hepatomegaly... DDs s/b= Portal HT, leukemia/lymphoma, HA including thalassemia/spherocytosis, storage ds, chr malaria...

CBC PBS LFT ESR RETIC COUNT awaited. I will post further progress.

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I totally agree with Dr.Manish. U can have lot of causes for splenimegaly but for MASSIVE solenomegaly u have very few causes especially at this age. My order would be 1. Storage disorder like GAUCHER 1 (check peripheral smear/ check for pulmonary / skeletal / neuro involvement ? Do either bone marrow to look for gaucher cells or enzyme assays to confirm diagnosis ) or NIEMANN PICK DISEASE (extra cherry red spot n failure to thrive ll be there) 2. If by any chance he S from Bihar or other endemic areas rule out KALA AZAR by doing RK 39 detection 3. PORTAL HYPERTENSION (EXTRA HEPATIC PORTAL VEIN OBSTRUCTION) though little rare at this age. But child would have developed hemoptysis n hepatomegaly goes against it. Another PHT which can present like this bit with hepatomegaly would be NON CIRRHOTIC PORTAL FIBROSIS (NCPF) but that s almost impossible at this age 4. HEMOLYTIC anemias. Here for thalassemia major(any severe hemolytic anemias) he S little older. He should have transfused at least once by one year. So I'm keeping this lower in the list. 5. At this age leukemias / lymphomas won't present as massive splenomegaly that too in an otherwise well looking child. Usually those kid would be sick looking n will have a palpable spleen at max that's it. So keeping at at the last step. KINDLY LET US KNOW THE PROGRESS.

Wat is weight and length of child...it seems chronic well compensated disease...as ther is no ascites and no blood transfusion...and echotexture is also normal...so RE cell infiltration is the cause I think...Gauchers ds and neiman pick are the possibility. ...is ther jaundice..pallor..bleeding...h o BT...bone pain..LN pathy...tropical spleno in endemic areas is also possible...pls let us knw CBC

Pls look I to chest .. CXR as fever 15 days ....TB pericarditis is dd

AML,CML lymphoma. Malaria. Immunodeficiency.

Differential diagnosis for hepatosplenomegaly& investigate


Adv. .hemogram with retic count and peripheral smear. .hb electrophoresis. .smear for mp. .widal. . Dd. .heriditary spherocytosis Hemolytic anemias.. Storage disorders Malaria

Agree with Dr. Manish Verma.

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