Al-Janazreh Hamdi, Abuzneid Yousef S, Khamayseh Iman, Morabito Fortunato, Alqam Bilal, Abusabbah Rosaline M F, Mustafa Fatima K, Sarahneh Shifa
Al-Quds University Faculty of Medicine, Jerusalem, Palestine.
Hematology Department and Bone Marrow Transplant Unit, Cancer Care Center, Augusta Victoria Hospital, Jerusalem, Palestine.
Ann Med Surg (Lond). 2021 Jul 14;68:102565. doi: 10.1016/j.amsu.2021.102565. eCollection 2021 Aug.
Chronic myelogenous leukemia (CML) is a chronic myeloproliferative disease characterized by a massive overproduction of myeloid cells. It is associated with the Philadelphia chromosome [Ph1, t (9; 22) (q34; q11)] or BCR-ABL fusion gene. CML usually undergoes a triphasic clinical course ending in a blast crisis, an accelerated phase of blasts and promyelocyte production. Ten percent of CML patients reach the blast crisis phase, with 20-30% of leukemias belonging to B-cell lymphoid lineage. However, a transformation of CML into T-cell acute lymphoblastic leukemia (T-ALL) is rare.
We present a 56-year-old male patient, known case of hypertension and Ph1 CML of eight years with a family history of Gaucher disease who developed T-ALL. The patient presented with lymphadenopathy and severe anemia, needing packed RBC transfusion, neutropenia and thrombocytopenia at the admission. However, the monocytes and basophils percentage were high. The patient underwent a cervical lymph node core biopsy, and the immunohistochemistry stains showed an invasion of neoplastic cells positive for CD3, CD5, BCL2, CD34, TdT and focally positive for C-Kit and negative for CD20, CD56 and pan-CK. These histopathology features were consistent with T-cell acute lymphoblastic leukemia (T-ALL).
Blast crisis remain a challenge in CML management. It's of great importance to do a full proper workup including lymph nodes biopsies. The aim is to reverse blast crisis and restore the chronic phase.
慢性粒细胞白血病（CML）是一种慢性骨髓增殖性疾病，其特征是髓细胞大量过度生成。它与费城染色体[Ph1，t（9；22）（q34；q11）]或BCR-ABL融合基因相关。CML通常经历一个三相临床过程，最终发展为急变期，即原始细胞和早幼粒细胞生成加速期。10%的CML患者会进入急变期，其中20 - 30%的白血病属于B细胞淋巴系。然而，CML转化为T细胞急性淋巴细胞白血病（T-ALL）的情况很少见。
我们报告一名56岁男性患者，有高血压病史，患Ph1 CML八年，有戈谢病家族史，发生了T-ALL。患者入院时表现为淋巴结病和严重贫血，需要输注浓缩红细胞，伴有中性粒细胞减少和血小板减少。然而，单核细胞和嗜碱性粒细胞百分比很高。患者接受了颈部淋巴结核心活检，免疫组化染色显示肿瘤细胞浸润，CD3、CD5、BCL2、CD34、TdT呈阳性，C-Kit局部呈阳性，CD20、CD56和泛细胞角蛋白呈阴性。这些组织病理学特征与T细胞急性淋巴细胞白血病（T-ALL）一致。
急变期仍然是CML治疗中的一个挑战。进行全面适当的检查，包括淋巴结活检非常重要。目的是逆转急变期并恢复慢性期。
