Hematoma or Bleeding As Initial Presentation of Chronic Myeloid Leukemia (CML): Review

Background: Chronic myeloid leukemia (CML) is defined per the WHO 2016 classification as a myeloproliferative neoplasm characterized by the chromosomal translocation t (9; 22) (q34.1; q11.2). Patients with CML can have a very wide range of symptoms at presentation. Most patients presents as an incidental finding on a routine complete blood count (CBC). Symptoms might include B symptoms like fever, loss of weight, and night sweats. While splenomegaly is the most common initial presentation in the chronic phase, in accelerated or blast phase of CML, the presentations might resemble acute leukemia with signs and symptoms of anemia, bleeding, petechiae, and ecchymosis. Bleeding might be the initial presentation of acute leukemia; however, it is scarce for CML, especially the chronic phase, to present initially with spontaneous bleeding without coagulopathy and thrombocytopenia. The mechanism behind this rare perennation is not known up to the moment.

Methods: A literature search was performed for English language articles using Medline data through PubMed, Scopus, and Google Scholar, for any date up to June 2022. The following keywords were used "CML," "spontaneous bleeding," "bleeding," and "hematoma.". We did not find any retrospective, prospective or clinical trials or reviews on this rare topic. We decided to review all the case reports and case series published in the literature and collect all the general characteristics, common findings, treatment, and outcomes

Results: Thirty articles with 30 patients were included. Most of them were males, 23 (76.7%) with a median age was 40 years, and the majority were of Asian origin 13 out of 17 (76.4%). Hematoma sites were different; intracranial was most affected in 11 (36.6%) patients, followed by intraabdominal and musculoskeletal (MSK) bleeding in 9 (30%) patients each. One case was reported as an intrathoracic bleed. All the patients diagnosed with CML chronic phase except one patient in accelerated phase without thrombocytopenia and one presented with blast crisis. Coagulopathy was reported in 2 patients only. The average duration of symptoms was around 6 days, and the median white blood cell count on presentation was 232,000, while 192,000 for platelets and 8.5 gm/dL for hemoglobin. The sequel reported were bilateral deafness in 1 patient, unilateral blindness in 1 patient, and one admission to the intensive care unit. Sixteen (53%) patients received imatinib 400 mg daily. Surgery was done in 12 (40%) patients, and a similar number were treated conservatively. Documented recovery was reported in 10 (33%) patients.

Overall survival was reported in 26 patients. 20 (66.6%) were alive, while 6 (33.3%) were dead. The cause of death was reported to be intracranial hemorrhage in 2 cases, sepsis in one case, and splenic rupture in one case.

Conclusion: Spontaneous bleeding or hematoma as an initial presentation of CML is rare. Thrombocytopenia and coagulopathy do not explain most of the cases. We hypothesize that male sex, Asian ethnicity, and hyperleukocytosis could be a risk factors.

No relevant conflicts of interest to declare.