Medicine · 2025
Lymphoplasmacytic lymphoma with IgG-λ and free λ light chain paraprotein: A case report.
СтатьяКлинический случайНазвание и аннотация: Перевод в очереди
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Wang Luting, Wan Qiuxia, Lu Lina, Cao Shuya, Liu Beibei, Wang Suyun
Аннотация
RATIONALE: Lymphoplasmacytic lymphoma (LPL) represents a rare, indolent form of B-cell neoplasm, with non-immunoglobulin M subtypes, including the immunoglobulin G (IgG)-λ variant, being notably uncommon. In this report, we document a case of LPL distinguished by the presence of monoclonal IgG-λ immunoglobulin and free λ light chains, alongside its distinctive molecular characteristics and therapeutic outcomes.
PATIENT CONCERNS: A 58-year-old male presented with fatigue, leukocytosis (75.45 × 109/L, 88.8% lymphocytes), lymphadenopathy, and splenomegaly. Serum studies detected an IgG-λ monoclonal protein (6.74 g/L) with concurrent elevation of free λ light chains. Bone marrow biopsy revealed marked hypercellularity, with lymphocytes comprising 80% of nucleated cells (predominantly plasmacytoid lymphocytes) and 5% plasma cell clusters. Genetic testing identified mutations in MYD88, CXCR4, and IGHV, along with trisomy 12 and del(13q14).
DIAGNOSES: He was diagnosed with LPL with IgG-λ monoclonal immunoglobulin and free λ light chains, classified as low risk per the Revised International Prognostic Scoring System.
INTERVENTIONS: The patient received 3 cycles of bendamustine plus rituximab therapy.
OUTCOMES: The blood cell count returned to normal and the spleen and lymph nodes were significantly reduced. Serum M protein levels decreased, and no obvious increase in B lymphocytes or plasma cells was found in the bone marrow. The patient achieved partial remission.
LESSONS: This case highlights the diagnostic and therapeutic challenges associated with the IgG-λ subtype of LPL, an uncommon variant of this rare malignancy. This report provides valuable insights into the clinical presentation, pathological features, molecular alterations, and treatment outcomes of this rare disease.
PATIENT CONCERNS: A 58-year-old male presented with fatigue, leukocytosis (75.45 × 109/L, 88.8% lymphocytes), lymphadenopathy, and splenomegaly. Serum studies detected an IgG-λ monoclonal protein (6.74 g/L) with concurrent elevation of free λ light chains. Bone marrow biopsy revealed marked hypercellularity, with lymphocytes comprising 80% of nucleated cells (predominantly plasmacytoid lymphocytes) and 5% plasma cell clusters. Genetic testing identified mutations in MYD88, CXCR4, and IGHV, along with trisomy 12 and del(13q14).
DIAGNOSES: He was diagnosed with LPL with IgG-λ monoclonal immunoglobulin and free λ light chains, classified as low risk per the Revised International Prognostic Scoring System.
INTERVENTIONS: The patient received 3 cycles of bendamustine plus rituximab therapy.
OUTCOMES: The blood cell count returned to normal and the spleen and lymph nodes were significantly reduced. Serum M protein levels decreased, and no obvious increase in B lymphocytes or plasma cells was found in the bone marrow. The patient achieved partial remission.
LESSONS: This case highlights the diagnostic and therapeutic challenges associated with the IgG-λ subtype of LPL, an uncommon variant of this rare malignancy. This report provides valuable insights into the clinical presentation, pathological features, molecular alterations, and treatment outcomes of this rare disease.