# Lymphocytic Lymphoma Transforming into Hodgkin Lymphoma in Sub-Saharan Africa: Case Report and Literature Review

**Authors:** Sokhna Aïssatou Touré, Dibor Niang, Serigne Mourtalla Gueye, Mohamed Keita, Alioune Badara Diallo, Elimane Seydi Bousso, Fatma Dieng, Blaise Felix Faye, Moussa Seck, Saliou Diop

PMC · DOI: 10.3390/hematolrep16030050 · Hematology Reports · 2024-08-05

## TL;DR

A rare case of lymphocytic lymphoma transforming into Hodgkin lymphoma is reported in Sub-Saharan Africa, highlighting diagnostic challenges in the region.

## Contribution

This is the first reported case of HvRS in Sub-Saharan Africa, emphasizing regional diagnostic limitations.

## Key findings

- The patient exhibited classic HvRS features, including Hodgkin and Sternberg cells alongside lymphocytic lymphoma.
- Diagnostic challenges were noted due to limited haematopathologists and technical resources in the region.
- EBV positivity and specific immunohistochemical markers were observed in the case.

## Abstract

The Hodgkin variant Richter syndrome (HvRS) is an infrequent complication occurring in 1% of lymphocytic lymphoma/chronic lymphocytic leukemia patients. We report a case of HvRS diagnosed in Sub-Saharan Africa. A 63-year-old patient was consulted for the investigation of an abdominal mass that had been evolving for 5 years prior to admission. His history revealed night sweats, 13% weight loss in 3 months and persistent pruritis. Examination revealed bilateral cervical axillary and inguinal macroadenopathies, painless abdominal distension, pruritic lesions and WHO 2 PS. The blood count showed anemia at 9.5 g/dL. Histology revealed a lymphomatous proliferation of diffuse architecture, nodular in places, with Hodgkin and Sternberg cells associated with small lymphocytes, histiocytes and eosinophilic polymorphs. Immunohistochemistry showed CD20, PAX5, BCL2, CD5, CD23 and MYC positivity; Ki67 at 10% and cyclin D1, BCL6 and CD10 negativity; CD30 positivity on Hodgkin and Sternberg cells that remained CD20 negative; difficulty interpreting CD15; EBV positivity (EBERs); and CD3 and CD5 positivity on reactive T cells. CD138 and kappa and lambda light chains were non-contributory. The extension work-up classified the patient as Ann Arbor stage III B with a Hasenclever score of 3/7. This case illustrates the difficulties in diagnosing HvRS in our countries, where the number of haematopathologists is insufficient and the technical facilities are limited.

## Linked entities

- **Proteins:** MS4A1 (membrane spanning 4-domains A1), PAX5 (paired box 5), BCL2 (BCL2 apoptosis regulator), CD5 (CD5 molecule), FCER2 (Fc epsilon receptor II), MYC (MYC proto-oncogene, bHLH transcription factor), Mki67 (antigen identified by monoclonal antibody Ki 67), ccnd1.S (cyclin D1 S homeolog), BCL6 (BCL6 transcription repressor), MME (membrane metalloendopeptidase), TNFRSF8 (TNF receptor superfamily member 8), FUT4 (fucosyltransferase 4), cd.3 (Cd.3 conserved hypothetical protein), CD5 (CD5 molecule), SDC1 (syndecan 1)
- **Diseases:** chronic lymphocytic leukemia (MONDO:0004948), Hodgkin lymphoma (MONDO:0004952), anemia (MONDO:0002280)

## Full-text entities

- **Genes:** CCND1 (cyclin D1) [NCBI Gene 595] {aka BCL1, D11S287E, PRAD1, U21B31}, TNFRSF8 (TNF receptor superfamily member 8) [NCBI Gene 943] {aka CD30, D1S166E, Ki-1}, BCL2 (BCL2 apoptosis regulator) [NCBI Gene 596] {aka Bcl-2, PPP1R50}, FCER2 (Fc epsilon receptor II) [NCBI Gene 2208] {aka BLAST-2, CD23, CD23A, CLEC4J, FCE2, FCErII}, PAX5 (paired box 5) [NCBI Gene 5079] {aka ALL3, BSAP, PAX-5}, 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}, SDC1 (syndecan 1) [NCBI Gene 6382] {aka CD138, SDC, SYND1, syndecan}, CD5 (CD5 molecule) [NCBI Gene 921] {aka LEU1, T1}, MYC (MYC proto-oncogene, bHLH transcription factor) [NCBI Gene 4609] {aka MRTL, MYCC, bHLHe39, c-Myc}, FUT4 (fucosyltransferase 4) [NCBI Gene 2526] {aka CD15, ELFT, FCT3A, FUC-TIV, FUTIV, LeX}, BCL6 (BCL6 transcription repressor) [NCBI Gene 604] {aka BCL5, BCL6A, LAZ3, ZBTB27, ZNF51}
- **Diseases:** Hodgkin and Sternberg (MESH:D005359), Hodgkin variant Richter syndrome (MESH:C537025), Lymphocytic Lymphoma (MESH:D015451), anemia (MESH:D000740), sweats (MESH:D013543), Hodgkin Lymphoma (MESH:D006689), pruritis (MESH:D011537), axillary and inguinal macroadenopathies (MESH:D006552), pruritic lesions (MESH:C535817), weight loss (MESH:D015431), abdominal distension (MESH:D000007)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

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## References

21 references — full list in the complete paper: https://tomesphere.com/paper/PMC11348024/full.md

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Source: https://tomesphere.com/paper/PMC11348024