# Successful Treatment of Leukemic Mature B-Cell Lymphoid Neoplasm with Similar Features to Splenic Marginal Zone Lymphoma Possessing Aberrant Myeloid Markers

**Authors:** Shinsaku Imashuku, Naoko Kudo, Kagekatsu Kubo, Katsuyasu Saigo

PMC · DOI: 10.1155/2015/181263 · Case Reports in Hematology · 2015-10-08

## TL;DR

A patient with a leukemic B-cell lymphoma resembling splenic marginal zone lymphoma was successfully treated with a combination of drugs without surgery.

## Contribution

Demonstrates successful treatment of a rare leukemic lymphoma with aberrant myeloid markers using non-surgical therapy.

## Key findings

- The patient showed complete remission for over 44 months after treatment.
- Combination therapies of rituximab with bendamustine and cladribine were effective.
- No M-protein was detected during remission.

## Abstract

In splenic marginal zone lymphoma (SMZL), there are cases that cannot accurately be classified as such because of overlapping morphologic and/or immunophenotypic features. We report here a 76-year-old Japanese female, who showed leukemic B-cell lymphoproliferative disease possessing characteristic features identified for SMZL. The patient was leukemic with white blood cell counts 49,400/µL (abnormal cells, 78.5%) and neoplastic cells were characterized by aberrant expression of myeloid markers with CD19+CD13+ (64.2%) and CD20+CD11c+ (25.1%). Considering her history of previous chemotherapy and systemic leukemic phase of the disease, we treated the patient without performing splenectomy, with successful use of a combination of rituximab/bendamustine hydrochloride and of rituximab/cladribine. The patient has been in a complete remission longer than 44 months, with no detectable M-protein.

## Linked entities

- **Proteins:** CD19 (CD19 molecule), ANPEP (alanyl aminopeptidase, membrane), MS4A1 (membrane spanning 4-domains A1), ITGAX (integrin subunit alpha X)
- **Chemicals:** bendamustine hydrochloride (PubChem CID 77082), cladribine (PubChem CID 20279)
- **Diseases:** splenic marginal zone lymphoma (MONDO:0019462)
- **Species:** Homo sapiens (taxon 9606)

## Full-text entities

- **Genes:** CD34 (CD34 molecule) [NCBI Gene 947], FCER2 (Fc epsilon receptor II) [NCBI Gene 2208] {aka BLAST-2, CD23, CD23A, CLEC4J, FCE2, FCErII}, BCL2 (BCL2 apoptosis regulator) [NCBI Gene 596] {aka Bcl-2, PPP1R50}, CCND1 (cyclin D1) [NCBI Gene 595] {aka BCL1, D11S287E, PRAD1, U21B31}, MYOM2 (myomesin 2) [NCBI Gene 9172] {aka TTNAP}, IL2RA (interleukin 2 receptor subunit alpha) [NCBI Gene 3559] {aka CD25, IDDM10, IL2R, IMD41, TCGFR, p55}, CRP (C-reactive protein) [NCBI Gene 1401] {aka PTX1}, IL2RB (interleukin 2 receptor subunit beta) [NCBI Gene 3560] {aka CD122, IL15RB, IMD63, P70-75}, CD5 (CD5 molecule) [NCBI Gene 921] {aka LEU1, T1}, HLA-G (major histocompatibility complex, class I, G) [NCBI Gene 3135] {aka MHC-G}, MME (membrane metalloendopeptidase) [NCBI Gene 4311] {aka CALLA, CD10, CMT2T, NEP, SCA43, SFE}, KRT20 (keratin 20) [NCBI Gene 54474] {aka CD20, CK-20, CK20, K20, KRT21}, CD19 (CD19 molecule) [NCBI Gene 930] {aka B4, CVID3}, ANPEP (alanyl aminopeptidase, membrane) [NCBI Gene 290] {aka AP-M, AP-N, APN, CD13, GP150, LAP1}, CD2 (CD2 molecule) [NCBI Gene 914] {aka LFA-2, SRBC, T11}, ITGAX (integrin subunit alpha X) [NCBI Gene 3687] {aka CD11C, SLEB6}, CD33 (CD33 molecule) [NCBI Gene 945] {aka CD33rSiglec, SIGLEC-3, SIGLEC3, p67}, PTPRC (protein tyrosine phosphatase receptor type C) [NCBI Gene 5788] {aka B220, CD45, CD45R, GP180, IMD105, L-CA}
- **Diseases:** lymph node swelling (MESH:D000072717), trisomy 18 (MESH:D000073842), myelofibrosis (MESH:D055728), toxicity (MESH:D064420), B-cell lymphocytosis (MESH:D015448), splenomegaly (MESH:D013163), mantle cell lymphoma (MESH:D020522), deletion (MESH:D002872), chromosome abnormality (MESH:D002869), 46, XX (20/ (MESH:D058489), trisomy 3 (MESH:D014314), Leukemic (MESH:D007938), hairy cell leukemia (MESH:D007943), Lymphoma (MESH:D008223), B-Cell Lymphoid Neoplasm (MESH:D016393), SMZL (MESH:D018442)
- **Chemicals:** bendamustine (MESH:D000069461), COP (-), uric acid (MESH:D014527), pirarubicin (MESH:C027260), purine (MESH:C030985), rituximab (MESH:D000069283), creatinine (MESH:D003404), cladribine (MESH:D017338), bilirubin (MESH:D001663)
- **Species:** Homo sapiens (human, species) [taxon 9606], Human immunodeficiency virus 1 (no rank) [taxon 11676]
- **Cell lines:** SMZL — Gallus gallus (Chicken), Transformed cell line (CVCL_6295)

## Full text

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## Figures

1 figure with captions in the complete paper: https://tomesphere.com/paper/PMC4617874/full.md

## References

9 references — full list in the complete paper: https://tomesphere.com/paper/PMC4617874/full.md

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Source: https://tomesphere.com/paper/PMC4617874