Khan Najeebullah, Raza Syed Shahmeer, Khan Uzma Ikhtiar, Hafez Irfanullah, Abdullah Mohammad Ammar, Hassan Masroor, Rukh Hijaz Asfandiar Shah, Hussain Amer Kamal, Nadeem Muhammad Daniyal
Northampton General Hospital, UK.
Department of Medicine, Hayatabad Medical Complex, Peshawar, Pakistan.
J Ayub Med Coll Abbottabad. 2019 Apr-Jun;31(2):272-275.
Plasma cell leukaemia (PCL) is a very rare plasma cell dyscrasia with a significant number of monoclonal plasma cells in the peripheral blood. It is diagnosed by the presence of ≥ 2x109 /L plasma cells in the blood or by plasma cells making up ≥20% of the leukocyte count. It can arise from a leukemic transformation of multiple myeloma, or more commonly it can be primary. Regardless of its origin, it carries a very dire prognosis. It responds very poorly to the traditional chemotherapy regimens used for multiple myeloma. We present the case of a 50 years old female who presented to our hospital with a complicated UTI and severe generalized body aches. She was diagnosed as a case of plasma cell leukaemia and was treated with cyclophosphamide and dexamethasone, however she failed to go into remission. Her condition deteriorated and she ultimately passed away 1.5 months after diagnosis. The recommended treatment for PCL is aggressive combination chemotherapy followed by stem cell transplantation. However, there is no consensus regarding the treatment of plasma cell leukaemia, and treatment should be individualized based on the patient profile. Once diagnosed, the prognosis is poor.
浆细胞白血病（PCL）是一种非常罕见的浆细胞发育异常疾病，外周血中存在大量单克隆浆细胞。通过血液中浆细胞≥2×10⁹/L或浆细胞占白细胞计数≥20%来诊断。它可由多发性骨髓瘤的白血病转化引起，或更常见的是原发性的。无论其起源如何，预后都非常严峻。它对用于多发性骨髓瘤的传统化疗方案反应很差。我们报告一例50岁女性，因复杂的尿路感染和严重的全身疼痛前来我院就诊。她被诊断为浆细胞白血病，并接受了环磷酰胺和地塞米松治疗，但未能缓解。她的病情恶化，最终在诊断后1.5个月去世。PCL的推荐治疗方法是积极的联合化疗，随后进行干细胞移植。然而，关于浆细胞白血病的治疗尚无共识，治疗应根据患者情况个体化。一旦确诊，预后很差。
