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Pontaneous Regression Of Cutaneous Blastic Plasmacytoid Dendritic Cell Neoplasm Followed By Acute Monocytic Leukemia Evolving From Myelodysplastic Syndrome

Yasuda et al., 2014Leukemia

Yasuda, H., Takaku, T., Tomomatsu, J., Hosone, M., Tanaka, H., & Komatsu, N. (2014). spontaneous regression of cutaneous blastic plasmacytoid dendritic cell neoplasm followed by acute monocytic leukemia evolving from myelodysplastic syndrome. Internal medicine (Tokyo, Japan), 53(23), 2717–2720. https://doi.org/10.2169/internalmedicine.53.2740

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Case Details

Disease Location

Skin, stomach

Personal Characteristics

67-year-old man

Clinical Characteristics

Presented with multiple erythematous nodules on the trunk and limbs. A skin biopsy revealed medium-sized blastoid cells infiltrating the dermis and subcutis but sparing the epidermis, diagnosis of blastic plasmacytoid dendritic cell neoplasm (bpdcn) was made. Upper gastrointestinal (gi) endoscopy revealed two polypoid lesions in the gastric corpus, he biopsy again revealed medium-sized blastoid cells infiltrating the lamina propria which showed the same marker expression pattern as the cutaneous bpdcn cells. A bone marrow (bm) evaluation revealed a blast count of 13.2% along with dysplastic features, such as micromegakaryocytes and nuclear hypolobation of granulocytes, suggesting concomitant mds. Was hospitalized in late march 2012 due to sudden hematemesis. Upper gi endoscopy showed multiple hemorrhagic ulcers in the gastric corpus, but a biopsy was not conducted due to the patient’s poor general condition. Evolution of mds to acute monocytic leukemia with morphology resembling acute myeloid leukemia-m5b was confirmed. He developed an acute immunerelated thrombocytopenia due to epatientifibatide, which required platelet transfusions.

Remission Characteristics

He skin lesions spontaneously regressed and complete diminishment

Treatment & Mechanisms

Proposed Remission Mechanisms

Not discussed

Clinical Treatment

Idarubicin hydrochloride (after recurrence)