De Novo CD5 Negative Blastic Mantle Cell Lymphoma Presented with Massive Bone Marrow Necrosis without Adenopathy or Organomegaly.
PMID 26347832 | PMCID PMC4546951 | DOI 10.1155/2015/146598 · Case reports in hematology · 2015 · View on PubMed ↗
The recent World Health Organization (WHO) classification defines mantle cell lymphoma (MCL) as a distinct entity characterized by a unique immunophenotype and a molecular hallmark of chromosomal translocation t(11;14)(q13;q32). We report an unusual case of an advanced stage of CD5 negative MCL with a blastoid variant with a massive bone marrow (BM) necrosis as an initial presenting feature, with no adenopathy or hepatosplenomegaly. The pathologic features showed blastoid variant of MCL and flow cytometry showed that the tumor cells were CD5-, CD19+, CD20+, FMC-7+, CD23-, and lambda light chain restricted. Chromosomal analysis, using karyotype and fluorescent in situ hybridization (FISH), demonstrated karyotypic abnormalities in addition to the t(11;14). Our case study may be reported as a unique case of CD5- blastic MCL with unusual presentation and findings which made the diagnosis of MCL difficult.
Validated evidence
| Type | Entity | Source evidence | Confidence | Extractor |
|---|---|---|---|---|
| gene | CD19 | “The pathologic features showed blastoid variant of MCL and flow cytometry showed that the tumor cells were CD5-, CD19+, CD20+, FMC-7+, CD23-, and lambda light chain restricted.” | 0.98 | hgnc_dict_v1 |
| phenotype | lymphoma | “De Novo CD5 Negative Blastic Mantle Cell Lymphoma Presented with Massive Bone Marrow Necrosis without Adenopathy or Organomegaly.” | 0.98 | phenotype_alias_lexicon_v2 |