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.
Keywords: braf v600e mutations; cladribine; hairy cell leukemia; spontaneous splenic rupture; unusual presentation of hairy cell leukemia.
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