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Haemophagocytic lymphohistiocytosis and off label use: case report A 6-year-old boy developed haemophagocytic lymphohistiocytosis (HLH) following treatment with peginterferon-alfa-2a for hepatitis-B virus (HBV) infection. Thereafter, he received off label treatment with ruxolitinib for the HLH [routes and duration of treatment to reaction onset not stated; not all dosages stated]. The boy was hospitalised due to high fever lasting for more than 1 month (current presentation). Laboratory tests showed anaemia, leukopenia, enlargement of the spleen, hypofibrinogenemia, hypertriglyceridemia, increased level of serum ferritin, high level interleukin-2 receptor (IL-2R) and elevated soluble CD251. His bone marrow puncture specimen showed haemophagocytosis. Based on the examinations, he was diagnosed with HLH as per the HLH-2004 protocol. Infectious workup showed positive result for HBV. During anamnesis, it was revealed that, he carried the HBV infection since birth. He was initially treated with vidarabine, unspecified antibiotics and immune-globulin [immunoglobulin] for the HBV infection, but the condition continued to progress. Thereafter, he was started on peginterferon-alfa-2a [pegylated interferon-a-2a] for 2 months due to persistently increasing HBVDNA and detection of abnormal liver function. Based on the evaluation of his medical history and medications, the development of fever was considered as secondary to the peginterferon-alfa-2a therapy. Hence, the man’s peginterferon-alfa-2a therapy was discontinued. No other possible triggers or HLH related mutations were identified. It was considered that a conventional therapy with glucocorticoids or cytotoxic chemotherapy might aggravate the HBVinfection. Therefore, he was started on off label treatment with ruxolitinib 2.5mg twice a day on hospitalisation day 2. After 3 days of treatment, his body temperature normalised. Additionally, entecavir was initiated on day 5 (started late due to unavailability in the hospital). Significant improvement was noted in his condition with the treatment; cytokines decreased after 6 days, WBC count and haemoglobin increased, and CRP normalised after 11 days. After one month, almost all clinical parameters normalised. Ruxolitinib therapy was continued for 3 months, and entecavir for 6 months. During follow up examination after 1 years, he was noted as normal without any adverse effects. Jianguo L, et al. Ruxolitinib in Alleviating the Cytokine Storm of Hemophagocytic Lymphohistiocytosis. Pediatrics 146: e20191301, No. 2, Aug 2020. Available from: URL: 803507320 http://doi.org/10.1542/peds.2019-1301

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Reactions 17 Oct 2020 No. 1826