Böhm J, Schaefer H E, Fisch P
Pathologisches Institut, "Ludwig-Aschoff-Haus", Albert-Ludwigs-Universität Freiburg, Postfach 214, 79002 Freiburg, Germany.
Pathologe. 2002 Nov;23(6):480-5. doi: 10.1007/s00292-002-0578-5. Epub 2002 Oct 9.
In 1986 we diagnosed chronic idiopathic myelofibrosis (CIMF) in a 45-year-old asymptomatic patient with hepatosplenomegaly. In 1996 splenectomy was performed because of hypersplenism, and chemotherapy with hydroxyurea was initiated. In 1999 generalised lymphadenopathy of chronic lymphocytic leukaemia (B-CLL) developed. A trephine biopsy showed leukaemic bone marrow infiltration. On heteroduplex analysis we found a clonal rearrangement of IgH in the leukaemic lymphocytes. The coincidence of chronic myeloproliferative and lymphoproliferative diseases in the same patient is a rare phenomenon. According to the relevant literature, seven cases with a combination of CIMF/CLL have been reported. Possible pathomechanisms for the development of such coincidences are: 1) a bilineage manifestation of a pluripotent stem cell proliferation, 2) independent proliferations of two distinct cell lines under a common leukaemogenic stimulus or 3) an accidental association. These coincidence cases often showed a mild clinical course and also in our case, the patient is still alive and in a stable disease condition 16 years after the initial diagnosis.
1986年,我们诊断出一名45岁无症状且伴有肝脾肿大的患者患有慢性特发性骨髓纤维化(CIMF)。1996年,因脾功能亢进进行了脾切除术,并开始使用羟基脲进行化疗。1999年,该患者出现了慢性淋巴细胞白血病(B-CLL)的全身性淋巴结病。骨髓活检显示有白血病骨髓浸润。通过异源双链分析,我们在白血病淋巴细胞中发现了IgH的克隆重排。同一患者同时出现慢性骨髓增殖性疾病和淋巴增殖性疾病是一种罕见现象。根据相关文献,已有7例CIMF/CLL合并病例的报道。这种合并情况发生的可能病理机制为:1)多能干细胞增殖的双系表现;2)在共同的致白血病刺激下两种不同细胞系的独立增殖;3)偶然关联。这些合并病例通常临床病程较轻,我们的病例也是如此,患者在初次诊断16年后仍然存活,病情稳定。