Hepatosplenomegaly, Lymph Nodes and Erythroid Hyperplasia

4th Pediatric Infectious Diseases Conference

ISSN 0973 - 0958

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Rakesh Kumar, Binay Ranjan, Manjul Vijay Department of Pediatrics, Katihar medical college, Katihar...

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	Specialist Answers
Question
I would be happy if someone helps in diagnosis of this problem. 13 yr old child presented to us with fever with chills she had recurrent episodes since her 9 yrs of age, every time requiring long duration of hospital stay. We got hepato splenomegaly(massive) and few temporary lymph nodes. Every work up for malaria, dengue, UTI, Cholangitis came negative. Then we proceeded with work up for connective tissue disorders which was also found negative. Bone Marrow showed Erythroid Hyperplasia. This time when she was admitted, she collapsed and went into picture of DIC. It was very difficult for us to revive her out of it. Brucella, Leishmaniasis work up was also negative. THe only positive report we got in the previous admission was TB PCR positivity in CSF. We started her empirically on ATT. We feel she has some immuno deficiency that makes her succumb to recurrent fatal infections. However her HIV Status was negative. Whole body gallium scan also showed nothing. At present she is out of ICU in the ward waiting for the next episode of infection. Can u help us in diagnosis? How to rule out immunodeficiency? We have sent samples for complement analysis and Immunoglobulin Electrophoresis and results are awaited. Work up of sarcoidosis is going on. What else to rule out?
Answer
What are her liver function tests and what is the ultrasonography report? Has a liver biopsy been considered to rule out storage, TB, sarcoid etc? What is her baseline CBC and ESR? Apart from immunodeficiency (at her age, most common would be common variable immunodeficiency), one needs to rule out autoimmune disorder. Also if she is from the northern states, kala azar should be rule out for which again liver biopsy would be more useful. Back to All Questions

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