Blastic Plasmacytoid Dendritic Cell Neoplasm with Pulmonary Involvement and Atypical Skin Lesion

Abstract

Patient: Female, 51Final Diagnosis: Blastic plasmacytoid dendritic cell neoplasmSymptoms: Pulmonary bleeding • small skin lesionMedication: Hyper-CVAD • methotrexate • cytarabineClinical Procedure: —Specialty: HematologyObjective:Rare diseaseBackground:Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematodermic malignancy neoplasm with highly aggressive course and poor prognosis. This disease typically presents with cutaneous involvement as the first manifestation, with subsequent or simultaneous spread to bone marrow and peripheral blood.Case Report:Here, we report the case of a 51-year-old woman who presented a violaceus skin lesion on the lateral region of the right thigh, weight loss, fever, and lymphadenopathies. Computed tomography (CT) displayed thoracic and abdominal lymph node and alveolar bleeding. Flow cytometry from circulating blastic cells was compatible with BPDCN (CD4+, CD56+ and CD123+). She underwent 5 cycles of hyper-CVAD alternating with high-dose methotrexate and cytarabine, but the patient died due to alveolar bleeding and sepsis.Conclusions:We report a rare case of BPDCN characterized by an aggressive course, presence of atypical skin lesion, a finding suggestive of pulmonary infiltration, and nonresponse to induction chemotherapy, leading to late diagnosis and therapeutic management. Because of the late recognition of the skin lesion, neoplastic cells infiltrated the dermis and spread as the disease progressed rapidly to a fatal course.

Rapidly progressing blastic plasmacytoid dendritic cell neoplasm causing diffuse skin thickening: A case report with sequential computed tomography examinations

“…Given that the patient was not receiving chemotherapy after the first discharge, changes from the initial CT to the follow-up CT seemed to be the natural history of BPDCN, which has been largely unknown. Other reported CT imaging findings of BPDCN include pulmonary ground-glass opacity with interlobular septal thickening, indicating diffuse alveolar hemorrhage, in a patient with BPDCN involving the lungs [5] , and hepatosplenomegaly resulting in atraumatic splenic rupture [13] .…”

“…CT has been mainly used to assess the extent and depth of the skin lesions, as well as the metastases in patients with BPDCN [4] , [5] , [6] , [7] , [8] , [9] , [10] , [11] , [12] , [13] , [14] . CT shows round and ovoid homogeneous cutaneous mass with soft tissue density with or without subcutaneous invasion as seen in the present case.…”

“…Rapid progression to the terminal leukemic phase is common, resulting in a short mean survival of 12–16 months [3] . To our knowledge, there have been at least 11 reports with CT findings [4] , [5] , [6] , [7] , [8] , [9] , [10] , [11] , [12] , [13] , [14] , including 4 18 F- fluorodeoxyglucose positron emission tomography / CT ( 18 F-FDG PET/CT) reports, associated with BPDCN; however, knowledge of chronological changes on sequential CT examinations have been limited [ 13 , 14 ]. We herein report a case of a patient with rapidly progressing BPDCN with a sequential evaluation on CT.…”

A misdiagnosed case of blastic plasmacytoid dendritic cell neoplasm experiencing multiple recurrences who underwent allogeneic stem cell transplantation: a case report

“…Unusual cutaneous and extracutaneous manifestations have been reported in some cases, including gingival lesions, acute rheumatic fever, pulmonary, central nervous system (CNS), and testicular infiltration [ 12 , 13 ]. Besides, neoplastic cells metastasize to peripheral blood and bone marrow, lymph nodes, and spleen in 60–90%, 56%, and 34–78% of cases, respectively.…”

Diagnosis, treatment, and genetic characteristics of blastic plasmacytoid dendritic cell neoplasm: A review

“…[3,15] In addition, very rarely, BPDCN may involve the gallbladder, lungs, breasts, kidneys, and bladder. [16][17][18][19] In conclusion, the clinical manifestations of BPDCN are varied, and the specificity is weak, resulting in missing or misdiagnosing the disease.…”