Hematology/oncology clinics of North America
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Hematol. Oncol. Clin. North Am. · Feb 2018
ReviewThe Peripheral Neuropathies of POEMS Syndrome and Castleman Disease.
Polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes (POEMS) syndrome is a rare paraneoplastic disorder. The polyneuropathy can be the presenting symptom and is typically a painful, motor-predominant polyradiculoneuropathy often mimicking chronic inflammatory demyelinating polyradiculoneuropathy. ⋯ Castleman disease (CD) is seen in a subset of these patients, and when present the neuropathy is similar but less severe. In contrast, in those patients with purely CD, the neuropathy is often a mild, painless distal sensory neuropathy.
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Unicentric Castleman disease (UCD) is a rare lymphoproliferative disorder that manifests typically as proliferation of a single lymph node or region of lymph nodes. Histologically, hyaline vascular variant is found in a majority of UCDs. UCD commonly presents in younger patient populations. ⋯ It is difficult to achieve a definitive diagnosis by imaging alone. Histologic examination of the lesion remains the gold standard for diagnosis. Complete surgical resection is the best primary treatment modality for UCD resulting in excellent long-term survival and low recurrence rates.
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The term Castleman disease encompasses several distinct lymphoproliferative disorders with different underlying disease pathogenesis, and clinical outcomes. It includes unicentric and multicentric diseases with limited versus significant systemic symptoms, respectively. ⋯ Accordingly, accurate clinical diagnosis of Castleman disease requires careful and thorough clinicopathologic correlation. An overview of the key histopathologic features of Castleman disease is presented.
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Castleman disease (CD) is a rare and heterogenous group of disorders sharing in common an abnormal lymph node pathology. CD comprises distinct subtypes with different prognoses. ⋯ Multicentric CD is classically associated with systemic symptoms and poorer prognosis. In this article, the authors review how to diagnose the disease, keeping in context the clinical findings, biochemical changes and complications associated with CD.
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Acute painful episodes are the most common reason for emergency department visits among patients with sickle cell disease (SCD). Early and aggressive pain management is a priority. Emergency providers (EPs) must also diagnose other emergent diagnoses in patients with SCD and differentiate them from vaso-occlusive crisis. ⋯ Administration of intravenous fluids should be used judiciously. Blood transfusion may be considered. Coordination of care with hematology is an important part of the effective emergency department and long-term management of patients with SCD.