Massive Hepatomegaly - Can it be acute lymphoid leukemia?

 
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Department of Pediatric Gastroenterology and Hepatology, B J Wadia Hospital for Children, Mumbai, India

Address for Correspondence: Himali Meshram, 54-Harihar nagar, Beltarodi road, Besa, Nagpur 440034, India. Email: gshimali@yahoo.com


Keywords: ALL, large liver, children

Clinical Problem :
Why did the child have normal blood counts inspite of metastatic liver involvement?

Why did the child have normal blood counts inspite of metastatic liver involvement?


Discussion :
Hepatosplenomegaly is common in leukemia and can be due to various factors such as infiltration of leukemic cells, viral hepatitis or drug-induced.1 However, it is usually associated with other hematological parameters such as cytopenias and fever. Isolated hepatosplenomegaly as a sole manifestation of leukemia is rare in the pediatric age group.2 Liver involvement has been reported to be a frequent finding in patients with ALL, and it can present from asymptomatic hepatomegaly with infiltration of lymphocytes to hepatitis to acute liver failure.3 Extramedullary tumor infiltration into the lymph nodes, liver, spleen, testicles, and central nervous system are common.3 One-third patients with leukemia have infection or fever at presentation and one-half of the patients present with lymphadenopathy, splenomegaly and hepatomegaly.2 Pathology of the liver shows diffuse infiltration of the leukemic cell as seen in our case. Liver biopsy can be helpful. Pathologically, the liver shows diffuse enlargement secondary to infiltration by leukemic lymphoblasts. In ALL and chronic leukemoid leukemia (CLL), the involvement of periportal spaces by neoplastic cells is common.2 Systemic chemotherapy is the treatment of choice for hepatic infiltration with hepatic dysfunction and cytogenetic analysis is important to predict outcome and selection of therapy in ALL. Treatment should be initiated promptly to avoid fatal outcome.2 Our patient had an atypical presentation of ALL. She presented with loss of weight and appetite and abdominal distension with normal blood parameters.

References :
  1. Farahmand F, Fallahi GH, Sadeghian M. Acute Hepatitis as a Manifestation of Acute Lymphoblastic Leukemia. Iranian J Pediatr Soc. 2010; 2: 44-46
  2. Cesur S, Topcuoglu P, Afiik O, Birengel S , Ozcan M. Acute Hepatic Failure in a Case of Acute Lymphoblastic Leukemia. Turk J Med Sci. 2004;34:275-279
  3. Heincelman M, Karakala N, Rockey DC. Acute Lymphoblastic Leukemia in a Young Adult Presenting as Hepatitis and Acute Kidney Injury. J Investig Med High Impact Case Rep. 2016; 4: 2324709616665866.

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