# A case report of lymphoplasmacytic lymphoma with spherocytosis

**Authors:** Tianyu Ma, Tingting Liu, Bolin Yuan, Liang Wang, Guoqiang Liu

PMC · DOI: 10.1515/biol-2025-1286 · Open Life Sciences · 2026-03-02

## TL;DR

A 74-year-old man with lymphoplasmacytic lymphoma and spherocytosis was successfully treated with zanubrutinib and rituximab.

## Contribution

This paper reports a rare case linking lymphoplasmacytic lymphoma with spherocytosis and its treatment outcome.

## Key findings

- The patient had lymphoplasmacytic lymphoma confirmed by bone marrow biopsy and immunohistochemistry.
- Spherocytosis was diagnosed via peripheral blood smear and osmotic fragility tests.
- Targeted therapy with zanubrutinib and rituximab stabilized the patient’s condition.

## Abstract

This article presents a case of a rare lymphoplasmacytic lymphoma (LPL) complicated by spherocytosis in a 74-year-old male. The patient reported progressive fatigue and anemia and had a medical history of type 2 diabetes, hypertension, and cerebral infarction. Laboratory tests indicated moderate anemia (hemoglobin 80 g/L) and a monoclonal increase in serum IgG. A bone marrow biopsy combined with immunohistochemistry confirmed the diagnosis of lymphoplasmacytic lymphoma (IgG-κ type, MYD88 L265P negative). A peripheral blood smear revealed an increase in spherocytes, a positive acidified glycerolysis test (AGLT50), abnormal erythrocyte osmotic fragility, and a negative direct antiglobulin test. Genetic screening for hereditary erythrocyte diseases showed no pathogenic variations. The patient’s condition stabilized following targeted therapy with zanubrutinib and rituximab (ZR regimen). This case underscores the complexity of diagnosing dual hematological anomalies, highlights the importance of multidisciplinary collaboration, and seeks to explore the potential pathophysiological link between LPL and spherocytosis, offering a reference for diagnosis and treatment in similar clinical scenarios.

## Linked entities

- **Chemicals:** zanubrutinib (PubChem CID 135565884)
- **Diseases:** lymphoplasmacytic lymphoma (MONDO:0000432), spherocytosis (MONDO:0000094), type 2 diabetes (MONDO:0005148), cerebral infarction (MONDO:0002679)

## Full-text entities

- **Genes:** SDC1 (syndecan 1) [NCBI Gene 6382] {aka CD138, SDC, SYND1, syndecan}, CD200 (CD200 molecule) [NCBI Gene 4345] {aka MOX1, MOX2, MRC, OX-2}, LPL (lipoprotein lipase) [NCBI Gene 4023] {aka HDLCQ11, LIPD}, SPTB (spectrin beta, erythrocytic) [NCBI Gene 6710] {aka EL3, HS2, HSPTB1, SPH2}, MYOM2 (myomesin 2) [NCBI Gene 9172] {aka TTNAP}, LCN2 (lipocalin 2) [NCBI Gene 3934] {aka 24p3, MSFI, NGAL, p25}, CD38 (CD38 molecule) [NCBI Gene 952] {aka ADPRC 1, ADPRC1, cADPR1}, TNF (tumor necrosis factor) [NCBI Gene 7124] {aka DIF, IMD127, TNF-alpha, TNFA, TNFSF2, TNLG1F}, KRT20 (keratin 20) [NCBI Gene 54474] {aka CD20, CK-20, CK20, K20, KRT21}, ITGAE (integrin subunit alpha E) [NCBI Gene 3682] {aka CD103, HUMINAE}, TNFRSF17 (TNF receptor superfamily member 17) [NCBI Gene 608] {aka BCM, BCMA, CD269, TNFRSF13A}, IL3RA (interleukin 3 receptor subunit alpha) [NCBI Gene 3563] {aka CD123, IL-3R-alpha, IL3R, IL3RAY, IL3RX, IL3RY}, CD5 (CD5 molecule) [NCBI Gene 921] {aka LEU1, T1}, MYD88 (MYD88 innate immune signal transduction adaptor) [NCBI Gene 4615] {aka IMD68, MYD88D, WM1}, FCER2 (Fc epsilon receptor II) [NCBI Gene 2208] {aka BLAST-2, CD23, CD23A, CLEC4J, FCE2, FCErII}, CD22 (CD22 molecule) [NCBI Gene 933] {aka SIGLEC-2, SIGLEC2}, OGA (O-GlcNAcase) [NCBI Gene 10724] {aka MEA5, MGEA5, NCOAT}, CD19 (CD19 molecule) [NCBI Gene 930] {aka B4, CVID3}, CD28 (CD28 molecule) [NCBI Gene 940] {aka IMD123, Tp44}, ANK1 (ankyrin 1) [NCBI Gene 286] {aka ANK, SPH1, SPH2, ankyrin-1}, MME (membrane metalloendopeptidase) [NCBI Gene 4311] {aka CALLA, CD10, CMT2T, NEP, SCA43, SFE}, IL6 (interleukin 6) [NCBI Gene 3569] {aka BSF-2, BSF2, CDF, HGF, HSF, IFN-beta-2}
- **Diseases:** hemolysis (MESH:D006461), erythrocyte membrane defects (MESH:C567520), hematological malignancies (MESH:D019337), hyperactivity (MESH:D006948), proteinuria (MESH:D011507), hereditary erythrocyte diseases (MESH:D030342), fatigue (MESH:D005221), red blood cell membrane defects (MESH:C535298), Spherocytosis (MESH:C567159), diabetes (MESH:D003920), malignant (MESH:D009369), renal insufficiency (MESH:D051437), hepatosplenomegaly (MESH:C535727), IgG-type multiple myeloma (MESH:D009101), cryoglobulinemia (MESH:D003449), erythrocyte morphological abnormalities (MESH:D012010), small lymphocytic lymphoma (MESH:D015451), hyperviscosity syndrome (MESH:D013577), chronic inflammation (MESH:D007249), plasma cell diseases (MESH:D007952), WM (MESH:D008258), diabetic nephropathy (MESH:D003928), organ damage (MESH:D000092124), B-cell lymphoma (MESH:D016393), kidney damage (MESH:D007674), type 2 diabetes (MESH:D003924), speech impairment (MESH:D013064), lymph node enlargement (MESH:D000072717), LPL (MESH:D008223), dizziness (MESH:D004244), bone destruction (MESH:D001847), marginal zone lymphoma (MESH:D018442), hypercalcemia (MESH:D006934), weight loss (MESH:D015431), bone marrow infiltration (MESH:D001855), cerebral infarction (MESH:D002544), cytopenia (MESH:D006402), hypertension (MESH:D006973), HS (MESH:D013103), abnormalities (MESH:D000014), anemia (MESH:D000740), AIHA (MESH:D000744)
- **Chemicals:** phospholipids (MESH:D010743), bilirubin (MESH:D001663), folic acid (MESH:D005492), creatinine (MESH:D003404), glucose (MESH:D005947), vitamin B12 (MESH:D014805), zanubrutinib (MESH:C000629551), bendamustine (MESH:D000069461), rituximab (MESH:D000069283), ZR (MESH:D015040), Bruton's tyrosine kinase inhibitors (-)
- **Species:** Homo sapiens (human, species) [taxon 9606]
- **Mutations:** L265P

## Full text

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## Figures

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## References

14 references — full list in the complete paper: https://tomesphere.com/paper/PMC12952209/full.md

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Source: https://tomesphere.com/paper/PMC12952209