A 7 year old with splenomegaly
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A 7 year old with splenomegaly
02/01/2014
02/01/2014
Dr Ira Shah
https://www.pediatriconcall.com/Journal/images/journal_cover.jpg
Dr Ira Shah.
Medical Sciences Department, Pediatric Oncall, Mumbai, India.
ADDRESS FOR CORRESPONDENCE
Dr Ira Shah, Medical Sciences Department, Pediatric Oncall, 1, B Saguna, 271, B St. Francis Road, Vile Parle {W}, Mumbai 400056. |
Clinical Problem
A 7 year old girl hailing from Mumbai born of third degree consanguineous marriage presented with fever 7 days back that lasted for 3 days. She was treated with antimalarials, antipyretics and iron supplements. However, her CBC showed neutropenia and she had pallor for which she was referred for further management. Her birth history, milestones and dietary history were normal. On examination, she had pallor with firm splenomegaly of 6 cm. Other systems were normal. Investigations showed:
• Hemoglobin = 4.8 gm/dl
• WBC count = 1,800/cumm [40% polymorphs, 55% lymphocytes, 3% eosinophils, 2% monocytes] which normalized in 3 days.
• Platelets = Adequate
• Reticulocyte count = 2%
• MCH = 14.1 pg, MCV = 48.2 fl, MCHC = 29.3 gm/dl.
• RDW = 23.3%
• Peripheral smear – hypochromia, microcytosis, anisocytosis.
• USG abdomen – splenomegaly with subcentimetric mesenteric lymphnodes. Colour doppler was normal
• OptiMAL test for Malaria – Negative
• Funduscopy – Normal
• X-Ray Chest – No osteopetrosis
• Liver function tests – Normal
• Bone marrow examination – Hypercellular marrow with erythroid hyperplasia. No storage disorder.
• Hemoglobin electrophoresis – Normal
• Osmotic fragility – Normal.
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What is the cause of splenomegaly? What further tests are required?
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Discussion
Dr Ira Shah:
This child has anemia and with splenomegaly of unknown cause. Though initially, she had neutropenia, it normalized in 3 days suggestive of a transient neutropenia most likely due to a viral infection since the child also had fever initially.
Regarding the anemia, peripheral smear and RBC indices are suggestive of iron deficiency anemia. Though her reticulocyte count is not high, the bone marrow examination has already started showing erythroid hyperplasia with the child being on hematinics and with electrophoresis being normal it rules out hemolytic disorder.
Thus, the part that remains unexplained is splenomegaly. With storage disorder, hemolytic anemia ruled out and colour doppler of abdomen showing no portal hypertension, the common causes have been ruled out. Infection such as malaria has also been ruled out. However, this child resides in an area endemic for malaria and hence one should rule out Tropical splenomegaly. Here a malaria IgM & IgG test would be required. In this child malaria IgM has positive even though OptiMAL and peripheral smear for malarial parasites were negative. She was treated with chloroquine for 6 weeks following which her splenomegaly regressed.
It is also called as big spleen disease. Recently it has been redefined and more appropriately termed as “hyperreactive malarial syndrome” (HMS).
Criteria for diagnosis
Major criteria:
1. Gross splenomegaly
2. Presence of antimalarial antibodies
3. Clinical and immunologic response to antimalarials.
Minor criteria:
1. Hepatic sinusoidal lymphocytosis
2. Normal cellular and humoral responses to antigenic challenge
3. Normal phytohemagglutination response
4. Hypersplenism
5. Lymphocytic proliferation
6. Familial occurrence.
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| Compliance with ethical standards |
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Funding: None
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Conflict of Interest: None
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Cite this article as:
Shah I. A 7 year old with splenomegaly. Pediatr Oncall J. 2005;2: 61.
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