Proskuriakova Ekaterina, Sakhuja Anuradha, Khan Sarah, Shrestha Dhan B, Khosla Pam
Internal Medicine Department, Mount Sinai Hospital, Chicago, USA.
Hematology and Oncology Department, Mount Sinai Hospital, Chicago, USA.
Cureus. 2023 Jun 9;15(6):e40180. doi: 10.7759/cureus.40180. eCollection 2023 Jun.
Hairy cell leukemia (HCL) represents a rare B-cell malignancy with 2% of all leukemias and should be differentiated from HCL-like conditions, including HCL-variant (HCL-V) and splenic diffuse red pulp lymphoma (SDRPL). HCL gets its name from the short, thin projections that look like hair on its cells. It is associated with a specific immunophenotypic profile, cytopenia, and splenomegaly. Spontaneous splenic rupture can be a symptom of hematological malignancy such as HCL and is a life-threatening acute emergency. Here, we present a case of a 37-year-old man who presented to the hospital with signs of acute peritonitis and acute anemia and was found to have atraumatic splenic rupture secondary to splenomegaly. He underwent emergent angiography, where the bleeding splenic vessel was identified, and the patient was successfully treated with embolization. Immunophenotypic profile revealed that B-cells were positive for CD11c, CD103, CD25, and CD5, for which he received five days of cladribine and achieved complete clinical remission.
毛细胞白血病(HCL)是一种罕见的B细胞恶性肿瘤,占所有白血病的2%,应与HCL样疾病相鉴别,包括变异型毛细胞白血病(HCL-V)和脾弥漫性红髓淋巴瘤(SDRPL)。HCL得名于其细胞上类似毛发的短而细的突起。它与特定的免疫表型特征、血细胞减少和脾肿大有关。自发性脾破裂可能是HCL等血液系统恶性肿瘤的症状,是一种危及生命的急性紧急情况。在此,我们报告一例37岁男性患者,他因急性腹膜炎和急性贫血症状入院,被发现因脾肿大导致非创伤性脾破裂。他接受了急诊血管造影,确定了出血的脾血管,患者通过栓塞治疗成功治愈。免疫表型特征显示B细胞CD11c、CD103、CD25和CD5呈阳性,为此他接受了五天的克拉屈滨治疗并实现了完全临床缓解。