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首页> 外文期刊>American Journal of Case Reports >Hemophagocytic Lymphohistiocytosis and Relapsing Polychondritis with Acute Myelogenous Leukemia: Case Report and Review of the Literature
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Hemophagocytic Lymphohistiocytosis and Relapsing Polychondritis with Acute Myelogenous Leukemia: Case Report and Review of the Literature

机译：血小杂淋巴细胞增多症和急性髓性白血病复发多档炎：案例报告和文献综述

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Patient: Female, 16-year-old Final Diagnosis: Hemophagocytic lymphohistiocytosis ? relapsing polychondritis Symptoms: Fever ? joint pain ? leukopenia Medication: — Clinical Procedure: — Specialty: Cardiology ? Critical Care Medicine ? Hematology ? General and Internal Medicine ? Pediatrics and Neonatology ? Rheumatology Objective: Rare disease Background: This case report describes rare disease entities with possible associations that include relapsing polychondritis, a rare disease with systemic manifestations characterized by bouts of inflammation in hyaline cartilage in multiple body sites, and hemophagocytic lymphohistiocytosis (HLH), another potentially life-threatening condition that occurs due to erratic activation of the immune system accompanied by pancytopenia. Both diseases constitute a real challenge to diagnose and treat. These entities, their associations, and treatment protocols and prognosis for them are highlighted. Case Report: A 16-year-old female presented with features and complications of both relapsing polychondritis (RP) and HLH including costochondritis, fever, splenomegaly, thrombocytopenia, and anemia. After admission to the intensive care unit, symptomatic management included paracetamol, intravenous fluids, prednisolone 60 mg orally, intravenous immune globlulin, and warfarin. Unfortunately, the patient developed acute myelogenous leukemia (FAB AML M5b) after a period of remission and died due to sepsis and multiorgan failure. Conclusions: HLH and RP are two rare diseases that can present together. Whether this malignant process (AML) is a cause or a result of these diseases is unknown. In the case presented here, the patient developed features of AML after a period of remission from RP and HLH. This case report may provide perspective on diagnosis and treatment for clinicians faced with similar patients.
机译：病人：女性，16岁的最终诊断：血液检淋巴咽喉菌症？复发多档炎症症状：发烧？关节痛 ？白细胞减少药物： - 临床手术： - 专业：心脏病学？批判性护理药？血液学？一般和内科？儿科和新生儿学？风湿病学目标：稀有疾病背景：本病例报告描述了具有可能的关联的罕见疾病实体，包括复发多档炎，一种罕见的疾病，其具有透明软骨中的炎症在多个身体部位中的炎症，以及血液活性淋巴管菌（HLH），另一个可能威胁危及危及危害性的疾病，其伴随着PancyTopenia的免疫系统的不稳定。这两种疾病都构成了诊断和治疗的真正挑战。这些实体，他们的协会和治疗方案和预后的突出显示。案例报告：一名16岁女性介绍复发多档炎（RP）和HLH的特征和并发症，包括Costochondritis，发烧，脾肿大，血小板减少和贫血。在入院后，症状管理包括扑热息痛，静脉内液，60毫克口服，静脉内免疫气素和华法林。不幸的是，患者在缓解后发育急性髓性白血病（Fab AML M5B），并因败血症和多功能衰竭死亡。结论：HLH和RP是两种可以一起出现的罕见疾病。这种恶性过程（AML）是原因还是这些疾病的结果是未知的。在这里呈现的情况下，患者在RP和HLH缓解后的AML的特征。本案例报告可提供临床医生面临着类似患者的诊断和治疗的视角。

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