English (United Kingdom)

https://curated-unify.zendy.io/wp-json/zendy-region/v1/featured_content/oa?rat=en

https://curated-unify.zendy.io/wp-json/zendy-region/v1/highlighted_journal/

Global: Zendy Plus annual

Presents the access of premium content as premium feature

Premium Content

Presents the keyphrase highlighting as premium feature

Keyphrase Highlighting

Presents the summarisation as premium feature

Summarisation

Insights

Presents the pdf analysis as premium feature

PDF Analysis

Presents the zaia usage as premium feature

ZAIA

Global: Zendy Tools annual

Global: Zendy Free Plan

Global: Zendy Tools monthly

Global: Zendy Plus monthly

A 78-year-old Japanese man presented to our hospital with multiple skin lesions, multiple lymphadenopathies and moderate splenomegaly (Figure 1). Hematological examination revealed a white blood cell (WBC) count of 13100/μL with 63% blasts, hemoglobin concentration of 12.2 g/dL and platelet count of 42000/μL. Blasts in the peripheral blood were medium in size and had scant grey-blue cytoplasm (Figure 2A). On flow cytometry analysis of peripheral blood, abnormal cells expressing CD2, CD4, CD7 and CD56 were observed. Based on these findings, we suspected leukemic transformation of lymphoma. Lymph node biopsy was performed and the specimen exhibited diffuse proliferation of large blastic cells (Figure 2B). These cells were positive for CD4, CD56, CD123 and TCL1 on immunohistochemistry (Figure 2C, D). Infiltration of these abnormal cells was also noted in the specimen from the skin lesion b i o p s y a n d b o n e m a r r o w. T h e c h r o m o s o m a l analysis demonstrated a normal karyotype. Taken together, a diagnosis of blastic plasmacytoid dendritic cell neoplasm (BPDCN) was established. Fourteen days after the first visit, he returned with fever, anemia and tenderness in the left upper quadrant. We assumed progression of the disease and planned chemotherapy. Two days after admission, the WBC count, including blasts, started to decrease. Computed tomography (CT) demonstrated hematoma around the spleen and hemorrhagic ascites (Figure 3), indicating mild atraumatic splenic rupture (ASR). Serum chemistry revealed the elevation of C-reactive protein (CRP) and uric acid up to 18.58 mg/dL and 11.6 mg/dL, respectively. These findings suggested that strong inflammation and tumor lysis occurred simultaneously after ASR. Chemotherapy was postponed and he was managed conservatively. Afterwards, he developed leukopenia together with slight improvement of skin lesions, but the mechanism of this phenomenon was unclear. His WBC count recovered gradually over 2 months, and blasts were finally undetectable in the peripheral blood. A slight reduction in abnormal blasts was noted in the bone marrow aspirate. Soon afterwards, blasts increased in the peripheral blood, and anemia, thrombocytopenia and skin lesions progressed. One month later, he was admitted again to receive chemotherapy. Three days before the scheduled date of chemotherapy, he developed fever and sudden pain in the left upper quadrant again. Subsequently, the WBC count began decreasing and hemorrhagic ascites was again detected on CT imaging (Figure 4A). The elevation of CRP and uric acid was also observed again. The skin lesions had visibly improved (Figure 4B) and the infiltration of abnormal blasts in the bone marrow markedly decreased. These findings Atraumatic splenic ruptures triggered both remission and death in a single case of blastic plasmacytoid dendritic cell neoplasm