Neurolymphomatosis (NL) is defined as the involvement of the peripheral nervous system in lymphocytic invasion. It is a very rare form of lymphoma that may occur as an initial presentation or recurrence. It affects various peripheral nervous structures and can therefore mimic disc-related nerve root pathology or compressive mononeuropathy. NL often occurs in malignant B-cell non-Hodgkin lymphomas. Notwithstanding its aggressiveness or intractability, NL should be discriminated from other neurologic complications of lymphoma. Herein, we present a case of primary NL as the initial presentation of diffuse large B-cell lymphoma (DLBCL) of the sciatic nerve. The patient presented with weakness and pain in his left leg but had no obvious lesion explaining the neurologic deficit on initial lumbosacral and knee magnetic resonance imaging (MRI). NL of the left sciatic nerve at the greater sciatic foramen was diagnosed based on subsequent hip MRI, electrodiagnostic test, positron emission tomography/computed tomography, and nerve biopsy findings. Leg weakness slightly improved after chemotherapy and radiotherapy. We report a case wherein NL, a rare cause of leg weakness, manifested as the initial presentation of primary DLBCL involving the sciatic nerve at the greater sciatic foramen.
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Background Hemophagocytic lymphohistiocytosis (HLH) is a rare but severe, life-threatening inflammatory condition if untreated. We aimed to investigate the etiologies, outcomes, and risk factors for death in children and adults with HLH.
Methods The medical records of patients who met the HLH criteria of two regional university hospitals in Korea between January 2001 and December 2019 were retrospectively investigated.
Results Sixty patients with HLH (35 children and 25 adults) were included. The median age at diagnosis was 7.0 years (range, 0.1–83 years), and the median follow-up duration was 8.5 months (range, 0–204 months). Four patients had primary HLH, 48 patients had secondary HLH (20 infection-associated, 18 neoplasm-associated, and 10 autoimmune-associated HLH), and eight patients had HLH of unknown cause. Infection was the most common cause in children (14/35, 40.0%), whereas neoplasia was the most common cause in adults (13/25, 52.0%). Twenty-eight patients were treated with HLH-2004/94 immunochemotherapy. The 5-year overall survival (OS) rate for all HLH patients was 59.9%. The 5-year OS rates for patients with primary, infection-associated, neoplasm-associated, autoimmune-associated, and unknown cause HLH were 25.0%, 85.0%, 26.7%, 87.5%, and 62.5%, respectively. Using multivariate analysis, neoplasm-induced HLH (p=0.001) and a platelet count <50×109/L (p=0.008) were identified as independent risk factors for poor prognosis in patients with HLH.
Conclusion Infection was the most common cause of HLH in children, while it was neoplasia in adults. The 5-year OS rate for all HLH patients was 59.9%. HLH caused by an underlying neoplasm or a low platelet count at the time of diagnosis were risk factors for poor prognosis.
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