Fever, hepatosplenomegaly, anemia, lymphadenopathy

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Fever, hepatosplenomegaly, anemia, lymphadenopathy

Author: Pediatric Oncall

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A nine-year-old boy presented with fever for 10 days, diarrhea, and vomiting for 1 week. For the above complaints, he was admitted and diagnosed with acute gastroenteritis and treated with IV antibiotics consisting of Ceftriaxone and Amikacin to which he responded. However, 2 days later he developed sudden onset breathlessness and cough and X-Ray Chest was suggestive of bilateral bronchopneumonia. He was thus referred for further management. On admission, he had tachycardia (HR = 120/min) with tachypnea (respiratory rate = 40/minute) with intercostal and subcostal retractions. He had hypotension (B.P. = 80/40 mm of Hg) and fever with an axillary temperature of 104oF. On systemic examination, he had bilateral crepitations in both inframammary regions. Other systems were normal. He was thus diagnosed with pneumonia with septicemia and treated with IV fluids, intravenous antibiotics (piperacillin & tazobactum), given ionotropic support and oxygen to which he responded. Oxygen and ionotropic support were removed after 3 days, however, his fever persisted. On further inquiry, there was a history of similar complaints 1 year back, and was found to have pleural effusion for which he was treated with antituberculous therapy for 6 months. There was no history of drinking unpasteurized milk though there was a history of a cat scratch on his face 1 year back. For his fever, he was investigated and the hemogram showed leucocytosis with the presence of toxic granules on peripheral smear [Hemoglobin = 11.6 gm/dl, WBC count = 26,900/cumm, 87% polymorphs, 13% lymphocytes] ESR was 79 mm at end of 1 hour. His renal and liver function tests were normal. Blood bactec culture, urine culture, stool culture were negative. His Widal, ELISA for HIV, Dengue IgM and Paul Bunnel were also negative. Echocardiography was normal and there were no vegetations. Repeat X-Ray Chest was normal. Ultrasound of abdomen showed mild hepatosplenomegaly. His serum immunoglobulins by immunoturbidometry showed hypergammaglobulinemia [S. IgA = 198 mg/dl, S. IgG = 2223 mg/dl and S. IgM = 61 mg/dl]. Autoimmune markers in form of RA factor, ANA, ds DNA were negative and C3 was normal. Brucella IgG was positive in titre of 1:160. He was then started on Doxycycline and Gentamicin to which he did not respond. Subsequently he developed anemia, hepatosplenomegaly and lymphadenopathy and WBC count increased to 46,000/cumm. He required repeated blood transfusions for the anemia. His Coomb’s test was negative. A liver function tests showed deranged Prothrombin Time and Partial Thromboplastin Time. Renal function tests revealed a high serum creatinine of 2.4mg/dl though his urine output was normal. Serum Ferritin was 54,000 IU/dl

What is the diagnosis?

Answer Discussion :

S

Sameh mohamed

0

malignancy

S

Sam Basem

0

leukemoid reaction

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Answer 1

In view of anemia, hepatic derangement with renal involvement one must suspect Macrophage Activation Syndrome. (MAS). is characterized by persistent fever, lymphadenopathy, hepatosplenomegaly, depression of one or more blood cells lines, low erythrocyte sedimentation rate (ESR), raised liver cell enzymes and abnormalities of clotting profile. It is though to be caused by excessive activation and proliferation of T lymphocytes and macrophages. MAS can occur in a heterogenous group of diseases ranging from infections or neoplasms, hematological conditions and rheumatic disorders. Measurement of serum ferritin is a useful indicator of disease activity. Bone marrow examination may show presence of hemophagocytes. In this child the bone marrow examination showed hemophagocytes confirming macrophage activation. Treatment consists of high dose corticosteroid or IVIG as it has a high mortality. This child was treated with IV Methyl Prednisolone to which his fever, anemia and deranged liver responded.

Fever, hepatosplenomegaly, anemia, lymphadenopathy

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