Igala Marielle, Bopaka Regis Gothar, Khtabi Wiam, Benchekroun Said, Quessar Asma
Hematology Department, CHU IBN Rochd, Faculty of Medicine and Pharmacy, Universite Ain Chock, Casablanca Morocco.
Pneumology Department, CHU IBN Rochd, Faculty of Medicine and Pharmacy, Universite Ain Chock, Casablanca Morocco.
Pan Afr Med J. 2014 Sep 18;19:39. doi: 10.11604/pamj.2014.19.39.4546. eCollection 2014.
Primary Plasma cell leukaemia (pPCL) is a rare plasma cell (PC) malignancy. The strict criteria for the diagnosis is an absolute PC number greater 2 × 10(9)/L or a plasmocytosis accounting for > 20% of the differential white cell count that does not arise from a pre-existing multiple myeloma. pPCL was associated with aggressive clinic-biological features. Primary Plasma cell leukaemia is more characterised by an extra medullar involvement such as hepatomegaly, splenomegaly, lymphadenopathy, lepto-meningeal infiltration or extramedullary plasmocytomas. The prognosis of pPCL is very poor. We report the case of a fifty eight year-old man directed to the haematology department for diagnosis of pPCL revealed by a thoracic plasmocytomas mimicking a thoracic neoplasm. The patient received chemotherapy including a classic treatment for multiple myeloma but developed a pulmonary embolism. This case illustrates an uncommon presentation of pPCL the difficulty treating by multiple myeloma chemotherapy.
原发性浆细胞白血病(pPCL)是一种罕见的浆细胞(PC)恶性肿瘤。其严格的诊断标准是绝对浆细胞数大于2×10⁹/L或浆细胞增多症占白细胞分类计数的>20%,且并非由先前存在的多发性骨髓瘤引起。pPCL与侵袭性临床生物学特征相关。原发性浆细胞白血病更以髓外受累为特征,如肝肿大、脾肿大、淋巴结病、软脑膜浸润或髓外浆细胞瘤。pPCL的预后非常差。我们报告了一例58岁男性患者,因模仿胸段肿瘤的胸段浆细胞瘤而被送往血液科诊断为pPCL。该患者接受了包括多发性骨髓瘤经典治疗在内的化疗,但发生了肺栓塞。本病例说明了pPCL的一种不常见表现以及多发性骨髓瘤化疗治疗的困难。