Cutaneous Presentation Preceding Acute Monocytic Leukemia:4Cases Report And Literature Review

Aleukemic leukemia cutis (ALC) is a discrete tumor of leukemic cells involving the skin, which may be prior to the onset in bone marrow and peripheral blood for months or years. It is often difficult to diagnose and with a dismal prognosis. Acute monocytic leukemia (AML-M5), a subtype of ALC, has the highest incidence rate and nonspecific symptoms.Objective:Aimed to analyze the clinical characteristics, histopathological changes and immunohistochemical changes of AML-M5to provide basis for early diagnosis and Meta Analysis.Methods:Comprehensively analyzed the clinical characteristics, histopathological changes and immunohistochemical changes of4cases of AML-M5and reviewed13cases in english and chinese literature.Results:Clinical characteristics:It occured most frequently in elderly people, which average age was65.24years old(our group was73.5literature after1986was62.69). There was no gender difference. Typical cutaneous lesions presented as multiple reddish or purple red(occasionally dark red or brown) papules, plaques, or nodules involving the trunk and extremities. It also could be purpuric rash or ecchymosis. There was no obvious self-conscious symptoms (only1patient had pain reported abroad.). Histopathological changes:A dense monomorphous cellular infiltrate involving the dermis and subcuffs with perivascular and periadnexal accentuation. A zone of uninvolved papillary dermis that separated normal epidermis from the underlying dermal infiltrate was observed. The minor cells were characterized by a large kidney-shaped or oval nucleus with one or more conspicuous nucleoli, and abundant pale, slightly eosinophilic cytoplasm. Immunohistochemical analysis on paraffin sections:The marker CD68, CD4,CD45RO, CD56, LCA were positive; marker CD3, CD20, CD30, CD34, CD117, CD123were negative in all cases. Most patients died within24months after onset of skin lesions.Conclusions:ALC occurs most frequently in elderly people. Typical cutaneous lesions present as multiple reddish or purple red papules, plaques, or nodules. There is no obvious self-conscious symptoms. Histopathological changes include a dense monomorphous cellular infiltrate involving the dermis and subcuffs with perivascular and periadnexal accentuation, a uninvolved zone between dermis and epidermal layer. Although immunohistochemical changes lack specificities, CD4,CD56in our4patients were strongly positive and the marker CD123were negative.Expression of CD4,CD56and lack of CD123may be the Immunological markers for AML-M5-LC.