Two Atypical Cases of Pediatric Lymphohistiocytosis

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Journal of Investigative Medicine





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Case Report Hemophagocytic Lymphohistiocytosis is often the top differential for pediatricians encountering an acutely sick child with lymphohistiocytosis. We present two cases in which lymphohistiocytoses were due to Rosai Dorfman and Systemic Lupus Erythematosis. Case 1 is a 7 month old male with bilateral large cervical adenopathy who had failed a 5-day course of IV antibiotics. Parapharyngeal node biopsy revealed epithelioid histiocyte clusters and rare neutrophils in a background of granulomatous inflammation. Cultured tissue was positive for Serratia Marscesens, concerning for Chronic Granulomatous Disease. Open biopsy of an enlarged occipital node revealed marked sinus histiocytosis. Large eosinophilic histiocytes displayed emperipolesis of lymphocytes and granulocytes. Epithelioid histiocyte clusters were present, suggesting poorly formed granulomatous inflammation. These findings were consistent with a diagnosis of Rosai-Dorfman. Normal lab results for CGD and HLH were ultimately obtained. Case 2 is a 17 year old male who presented with a 30 lb weight loss over 2 months, 3 weeks of daily fever, sore throat, cough, dizziness, progressive weakness, and syncope. On exam he had post-cervical and axillary lymphadenopathy. Lymph node biopsy showed paracortical expansion with histiocytic cells. Lymphoid tissue displayed large geographic necrosis, karyorrhectic debris, and reactive histiocytes rimming necrotic areas. No neutrophils were seen. There were no pathogenic organisms. Interpretation was consistent with Kikuchi Disease, or histiocytic necrotizing lymphadenitis. Symptoms progressed, meeting 5 of 11 clinical criteria for the diagnosis of SLE, with oral ulcers, nonerosive arthritis, neurologic features, leukopenia 2 occasions, and anti-Smith antibody. ANA was negative. Pathological description of lymphadenopathy is essential in differentiating among varied histiocytic diseases. Pediatric lymphohistiocytic diseases have a broad differential diagnosis including lymphoma, HLH, CGD, Rosai Dorfmann, Kicuchi Syndrome and SLE. Accurate diagnosis of histiocytic lymphadenopathies is vital to appropriate treatment and prognosis of pediatric disorders.





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