Introduction: Primary Plasma Cell Leukemia (pPCL) is Plasma cell dyscrasia subtype which is rare and aggressive. It carries very poor prognosis.
It has unique clinical and laboratory prole. Its rst clinical presentation is leukemia. Peripheral blood examination shows circulating mature
looking yet clonal, plasma cells. On molecular and cytogenetic examinations, many aberrations are seen which are unique and make it a distinct
entity different from traditional Multiple Myeloma (MM).
Case presentation: 37 yr old Indian female presented with difculty in breathing for last 3 months and was initially evaluated for cardiac function
& COVID-19 screening. Peripheral blood examination revealed circulating plasma cells. Bone marrow apirate conrmed the initial diagnosis of
pPCL. She received BIODRONATE + Inj. BORTEZOMIB + Inj. CYCLOPHOSPHAMIDE + Tab Dexa and was advised for PETscan and skeletal
survey. But due to nancial constraints, family decided to go for complete systemic workup in next phase of chemotherapy cycle. She was
discharged with advise to be in close follow up and to complete her treatment cycles.
Discussion: pPCL needs to be diagnosed promptly to formulate optimal intensive therapy. This atypical presentation with shortness of breath of
rare entity of pPCLin such young age emphasizes the need for quick and thorough initial workup.
Conclusion: Because of rarity of this disease, there is paucity of literature from India and especially the impact of the standard therapies in resource
poor countries. Our case report highlights these challenges for conclusive management of this rare entity
Spontaneous splenic rupture in plasma cell leukemia
