Intracerebral hemorrhage as the first symptomatic manifestation of chronic myeloid leukemia (chronic phase): A case report and literature review

Abstract

Background: Chronic myeloid leukemia (CML) is mostly asymptomatic at diagnosis. Intracerebral hemorrhage (ICH), as the first presentation of CML in its chronic phase (CP) has only once been reported in the literature. In addition, CML (CP) patients developing ICH are equally rare, with only eight cases reported. ICH is more commonly associated with CML progressing to its end stage (accelerated phase [AP] and blast crisis [BC]). The pathophysiology of ICH in CML-CP is postulated to be due to leukostasis, unlike in the CML-AP/BC, where thrombocytopenia and coagulopathy are the underlying mechanisms. This case adds to the scarce literature on a rare and challenging complication of ICH in CML-CP, especially as these patients tend to rebleed and management is uncertain.

Case description: A 22-year-old male presented with a 2-week history of headaches and vomiting, associated with a 1-week history of the left-sided weakness. Initial blood work revealed hyperleukocytosis. The patient was investigated for CML with intracranial involvement. During his stay, his Glasgow coma score (GCS) dropped (from 14 to 11), prompting an urgent CT scan which revealed a large resolving ICH with perifocal edema and midline shift. A decompressive hemicraniectomy with expansion duraplasty was performed to alleviate the mass effect and reduce intracranial pressure. Three hours postoperatively, the patient developed an extradural hematoma which needed prompt evacuation. A postoperative CT revealed an improved midline shift, and after 7 days, his GCS improved to 15, and he began oncological treatment. Neurological symptoms were experienced by our patient at presentation with hyperleukocytosis on full blood count, which may implicate leukostasis as an underlying mechanism.

Conclusion: Even in the CP, CML patients presenting with mild neurological symptoms should be investigated to exclude intracranial bleeds. As these patients tend to rebleed, they should be conservatively managed unless there is a need to alleviate intracranial pressure.

Keywords: Chronic myeloid leukemia; Chronic phase; Hyperleukocytosis; Intracerebral hemorrhage; Intracranial involvement; Leukostasis.

Figure 1:

Preoperative CT scan.

Figure 2:

Postoperative CT scan (48 h).