Ong'ondi Matilda, Kagotho Elizabeth
Department of Internal Medicine; Hemato-oncology Unit, Kenyatta National Hospital, Nairobi, Kenya.
Department of Pathology and Laboratory Medicine, Aga Khan University hospital, Nairobi, Kenya.
Case Rep Hematol. 2021 Jul 27;2021:4843818. doi: 10.1155/2021/4843818. eCollection 2021.
Plasma Cell Leukemia (PCL) is a rare and aggressive form of plasma cell dyscrasia that can arise either de novo (primary plasma cell leukemia) or evolve from previously diagnosed and treated multiple myeloma (secondary PCL). We highlight three clinical cases with very different presentations as a reminder of this diagnosis. The cases also highlight the diversity and variability that cover a patient's journey that is highly dependent on accessibility based on financial capability and social support. The clinical presentation is more aggressive due to the higher tumour burden and more proliferative tumor cells with cytopenias being profound and more organomegaly. The diagnosis is made based on at least 20% of total white blood cells being circulating plasma cells with a peripheral blood absolute plasma cell count of at least 2 × 10/l. Treatment with novel agents followed by autologous stem cell transplant in those who are transplant eligible leads to better outcomes.
浆细胞白血病(PCL)是一种罕见且侵袭性强的浆细胞发育异常形式,可原发出现(原发性浆细胞白血病),也可由先前诊断和治疗过的多发性骨髓瘤演变而来(继发性PCL)。我们重点介绍三例临床表现差异很大的病例,以提醒大家注意这种诊断。这些病例还凸显了患者病程的多样性和变异性,这高度依赖于基于经济能力和社会支持的可及性。由于肿瘤负荷更高、肿瘤细胞增殖性更强,导致血细胞减少严重且脏器肿大更多,其临床表现更为侵袭性。诊断依据是循环血浆细胞至少占白细胞总数的20%,外周血绝对浆细胞计数至少为2×10⁹/L。对于符合移植条件的患者,采用新型药物治疗后进行自体干细胞移植可带来更好的治疗效果。