Bumps of blood cells: Blastic Plasmacytoid Dendritic Cell Neoplasm in an elderly Filipino
- Author:
Christian Bernard T. Cheng
1
;
Ellen M. Gatchalian
1
;
Flordeluna Z. Mesina
1
;
Alejandro Arevalo
2
Author Information
- Publication Type:Case Reports
- Keywords: Blastic plasmacytoid dendritic cell neoplasm; Cutaneous nodules; Case report
- MeSH: Leukemia, Myeloid, Acute
- From: Philippine Journal of Internal Medicine 2019;57(1):34-38
- CountryPhilippines
- Language:English
-
Abstract:
Introduction:Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematologic malignancy derived from the precursors of plasmacytoid dendritic cells. This malignancy presents with various noticeable cutaneous lesions and usually occurs in elderly males. Cutaneous manifestations usually precede leukemic dissemination to the lymph nodes, bone marrow, and peripheral blood which is associated with poor prognosis.
Case presentation:We present a case of a 60-year-old Filipino male with a four-month history of multiple hyperpigmented, reddish brown, firm, fixed, non-tender cutaneous nodules on the extremities, trunk, chest, and face. Two large masses was also noted on the left arm and left upper back..Tissue biopsy of the cutaneous mass showed Immunohistochemical stain findings positive for LCA, CD68, CD4, CD56, and CD123 which are compatible with BPDCN. Patient was initially asymptomatic with relatively normal blood count and was treated supportively but serial blood count monitoring showed worsening with progression to acute myelogenous leukemia. Patient was then started on the 7+3 protocol of cytarabine and idarubicine which provided flattening of the cutaneous nodules and improvement of blood counts. However, due to complications of the disease and the treatment, the patient succumbed to severe pulmonary infection and sepsis.
Discussion:Due to the varied, non-specific cutaneous manifestations and the similarity in the morphology of the skin lesions with other cutaneous conditions along with the rarity of this disease, there is difficulty in establishing the diagnosis of BPDCN as well as standardizing its treatment. Immunohistochemical stains play an important role in confirming the diagnosis as well as ruling out other differential diagnoses to tailor appropriate treatment.
Conclusion:Blastic plasmacytoid dendritic cell neoplasm (BPDCN) generally has a poor prognosis owing to the rapidity of its spread to the bone marrow and peripheral blood. Early diagnosis is essential to initiate early therapy and prevent progression. - Full text:FINAL_2018-016_Bumps.pdf
