[Blastic plasmacytoid dendritic cell neoplasm - a rare differential diagnosis of neoplastic skin infiltrations associated with systemic symptoms].
METHODS
A 70-year-old female patient developed a non-pruritic, indolent rash associated with infections and peripheral blood abnormalities. A skin biopsy was suggestive of malignant lymphoma of the skin.


METHODS
The peripheral blood count showed mild pancytopenia and 12 % blasts. Additional immunohistochemistry of the skin specimen as well as cytologic and flow cytometric examination of the bone marrow revealed an immature cell population expressing CD4 and CD56 which infiltrated both the dermis and, with an infiltration grade of about 85 %, the bone marrow.


METHODS
Blastic plasmacytoid dendritic cell neoplasm (formerly known as blastic NK cell lymphoma).


METHODS
After the first course of induction chemotherapy with daunorubicin and cytarabin, both the rash and the hematologic findings of bone marrow and peripheral blood showed a complete remission.


CONCLUSIONS
The blastic plasmacytoid dendritic cell neoplasm is a rare, aggressive hematopoietic neoplasm most likely related to acute myeloid leukemia (AML). Since cutaneous involvement is regularly present at diagnosis, the differential diagnosis of unexplained skin lesions should include this disease entity, especially if peripheral blood abnormalities are present. Despite the initial response to cytostatic therapy being mostly excellent, the prognosis is poor. Hence, treatment as high-risk AML seems advisable.
Löffler
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Klinische Kooperationseinheit Molekulare Hämatologie/Onkologie, Deutsches Krebsforschungszentrum, Im Neuenheimer Feld 581, 69120 Heidelberg.
Kosely
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F
Ho
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AD
Krämer
A
A
D000368:Aged; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001706:Biopsy; D001853:Bone Marrow / Q000473:pathology; D003937:Diagnosis, Differential; D005076:Exanthema; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D015470:Leukemia, Myeloid, Acute / Q000145:classification / Q000175:diagnosis* / Q000188:drug therapy; D010198:Pancytopenia; D011379:Prognosis; D012074:Remission Induction; D012867:Skin / Q000473:pathology*; D012878:Skin Neoplasms / Q000145:classification / Q000175:diagnosis* / Q000188:drug therapy
D000970:Antineoplastic Agents