Abstract
RATIONALE: Primary plasma cell leukemia is a rare and highly aggressive malignancy of the blood system, with rapid disease progression and a high early mortality rate. Currently, there is no recognized therapeutic regimen, leading to the adoption of strategies typically utilized for multiple myeloma, which, however, exhibit limited efficacy. Selinexor is considered effective in treating relapsed/refractory multiple myeloma, but there are currently no reports on its application in primary plasma cell leukemia. Here, we reported a case of primary plasma cell leukemia with multiple high-risk genetic factors (including 1q21+, 17p-, and 13q-) who received a chemotherapy regimen including selinexor, pomalidomide, and dexamethasone. PATIENT CONCERNS: This case was a 58-year-old male presenting with lower back pain, abdominal pain, and various systemic symptoms. DIAGNOSES: The initial diagnosis of intestinal obstruction at a local hospital was followed by a referral to our emergency department due to abnormal blood test results indicative of a hematologic disorder. Further investigations confirmed a rare diagnosis of primary plasma cell leukemia of the IgA-k light chain subtype. INTERVENTIONS: The patient was promptly treated with a chemotherapy regimen comprising selinexor, pomalidomide, and dexamethasone in addition to supportive care. OUTCOMES: Subsequent assessments showed a significant response to treatment, with improvement in symptoms, normalization of blood parameters, and achievement of very good partial response. However, due to financial constraints, the patient declined hematopoietic stem cell transplantation and eventually opted to discontinue treatment, leading to disease progression. LESSONS: The combination of selinexor with pomalidomide and dexamethasone has shown good efficacy in primary plasma cell leukemia with high-risk genetic abnormalities. Our case may provide evidence for developing an effective selinexor-based regimen for treating primary plasma cell leukemia with high-risk genetic abnormalities.