Khieu Michelle, Beauvais Alexis, Matz Rebecca, Bersabe Adrian, Brown Patrick, Brown Alexander, Fillman Eric, Hall Jordan
From the Departments of Pathology, Internal Medicine, and Hematology/Oncology, San Antonio Military Medical Center, Fort Sam Houston, and Dermatology, San Antonio Uniformed Services Health Education Consortium, Lackland Air Force Base, Lackland, Texas.
South Med J. 2017 Apr;110(4):308-313. doi: 10.14423/SMJ.0000000000000634.
Myeloid leukemia cutis (MLC) is a rare disease characterized by the infiltration of neoplastic myeloid, myelomonocytic, or monocytic precursors into the skin, producing clinically nonspecific-appearing papules, nodules, plaques, or purpura that necessitate biopsy for definitive diagnosis. In general, it is considered an extramedullary manifestation of acute myeloid leukemia (AML) similar to myeloid sarcoma, also known as a chloroma. MLC often develops in patients with an established diagnosis of AML and is only rarely seen before the onset of systemic disease. Even more rarely, some patients are found to have an underlying nonleukemic bone marrow disease, such as a myelodysplastic syndrome (MDS). It is in these latter settings that the somewhat perplexing terminology aleukemic leukemia cutis (ALC) has been used widely. As may be imagined, establishing a diagnosis of ALC can be problematic and typically requires a high index of suspicion with careful integration of clinical, histopathological, and laboratory data. Once a diagnosis of ALC is made, a complete hematologic workup, including assessment of the patient's bone marrow, is essential because a diagnosis of MLC (with or without systemic leukemia) generally indicates a poor prognosis. When discovered in a patient with MDS, MLC has been suggested to herald progression toward more advanced disease. We present a rare case of so-called aleukemic myeloid leukemia cutis in an 82-year-old man subsequently diagnosed as having MDS without progression to overt AML. This case also illustrates a seemingly rare Kikuchi disease-like inflammatory pattern that obscured the underlying leukemic infiltrate in the skin, delaying its recognition.
皮肤髓系白血病（MLC）是一种罕见疾病，其特征为肿瘤性髓系、髓单核细胞或单核细胞前体细胞浸润皮肤，产生临床上外观无特异性的丘疹、结节、斑块或紫癜，需要进行活检以明确诊断。一般来说，它被认为是急性髓系白血病（AML）的髓外表现，类似于髓系肉瘤，也称为绿色瘤。MLC常发生于已确诊AML的患者，在系统性疾病发作前很少见。更罕见的是，一些患者被发现患有潜在的非白血病性骨髓疾病，如骨髓增生异常综合征（MDS）。正是在这些情况下，“无白血病性皮肤白血病”（ALC）这一有些令人困惑的术语被广泛使用。可以想象，确立ALC的诊断可能存在问题，通常需要高度怀疑，并仔细整合临床、组织病理学和实验室数据。一旦做出ALC的诊断，完整的血液学检查，包括对患者骨髓的评估，至关重要，因为MLC（无论有无系统性白血病）的诊断通常预示着预后不良。当在MDS患者中发现时，MLC被认为预示着疾病向更晚期进展。我们报告一例82岁男性的罕见病例，其被诊断为所谓的无白血病性皮肤髓系白血病，随后被诊断为MDS，未进展为明显的AML。该病例还显示了一种看似罕见的类似菊池病的炎症模式，掩盖了皮肤中潜在的白血病浸润，延迟了对其的识别。
