Cutaneous plasmablastic lymphoma

While primary cutaneous diffuse large B-cell lymphoma leg type is the prototype of primary cutaneous high-grade B-cell lymphoma, there are rare forms with unusual features. This chapter covers cutaneous intravascular large B-cell lymphoma and cutaneous plasmablastic lymphoma.

Gene Expression Analysis Of Plasmablastic Lymphoma Identifies Down Regulation Of B Cell Receptor Signaling and Additional Unique Transcriptional Programs

Dramatic treatment response of cutaneous plasmablastic lymphoma in an HIV patient: a case report

Plasmablastic lymphoma (PBL) occurs mainly in immunocompromised individuals, usually secondary to human immunodeficiency virus (HIV) infection. It classically occurs intraorally, but has been described in extraoral locations. The aim of this study was to define the immunophenotype and Epstein-Barr virus (EBV) status in a large single-centre cohort of extraoral PBL (EPBL) in South Africa, a high-prevalence HIV setting. This retrospective study of 45 EPBLs included patients' age, gender, race, HIV status, and site. Cases were reviewed histologically, and classified morphologically as pure plasmablastic or plasmablastic with plasmacytic differentiation, and assessed immunohistochemically with antibodies against CD45, CD20, CD79a, PAX5, CD138, MUM1/IRF4, BLIMP1, VS38c, Ki67, bcl-6, CD10, cyclin D1, and human herpesvirus-8, by the use of standard automated procedures. EBV was assessed by the use of chromogenic in-situ hybridisation. Tumours were assessed with a fluorescence in-situ hybridisation (FISH) MYC break-apart probe. Twenty-seven PBLs showed pure plasmablastic morphology, and 18 showed plasmacytic differentiation. The male/female ratio was 1.5:1. The anus was the favoured extraoral site (31.1%), followed by lymph nodes (15.6%). All 29 patients with known HIV status were HIV-positive. The immunohistochemical profile recapitulated that reported for oral PBLs and EPBLs in HIV-positive and HIV-negative patients. EBV was positive in 92.5% of PBLs. FISH analysis showed MYC rearrangement in 48% of cases. This study showed a strong association of EPBLs with HIV and EBV infection, similarly to the previously described oral PBL. The strong EBV association together with other clinicopathological parameters and an immunohistochemical profile that includes CD45, CD20, MUM1/IRF4, CD138 and Ki67 may be used in distinguishing PBL from diffuse large B-cell lymphoma and plasma cell myeloma.

Actualización en procesos linfoproliferativos cutáneos relacionados con el virus de Epstein Barr: revisión sistématica

Primary cutaneous follicular center cell lymphomas represent a distinct type of cutaneous B-cell lymphoma, clinically characterized by localized skin lesions on the head or trunk and an excellent prognosis. Histologically similar lymphomas may occur on the legs. The clinical behavior of this group is still undefined, and controversy exists whether these lymphomas should be classified as follicular center cell lymphoma or B-immunoblastic lymphoma. We reviewed the clinical, histologic, and follow-up data of 18 patients with primary cutaneous large B-cell lymphoma of the legs. Primary cutaneous large B-cell lymphoma of the legs generally occurred in elderly patients (median age at diagnosis, 76 years), in particular women (male-female ratio, 7:2), and preferentially affected the lower legs (14 of 18 patients). Radiotherapy and/or systemic polychemotherapy resulted in complete remissions in 16 of 17 patients. Follow-up data demonstrated estimated 2- and 5-year survival rates of 77% and 58%, respectively. Histologic evaluation showed diffuse dermal infiltrates with variable proportions of centroblasts (large noncleaved cells), large centrocytes (large cleaved cells), and B immunoblasts. Seventeen of 18 patients were diagnosed as having primary cutaneous follicular center cell lymphoma; only 1 patient, whose histologic examination showed more than 30% immunoblasts, was diagnosed as having B-immunoblastic lymphoma. Primary cutaneous large B-cell lymphoma of the legs is a distinct clinicopathologic entity that mainly affects elderly patients and has an intermediate prognosis. Although most cases have a follicular center cell origin, primary cutaneous large B-cell lymphoma is proposed as the most appropriate term for this type of cutaneous lymphoma.

Primary cutaneous plasmablastic lymphoma revealing clinicallyunsuspected HIV infection*

The increasing incidence of primary cutaneous B-cell lymphomas (PCBCLs) presents new challenges for clinicians. Despite advances in the clinical and pathologic characterization of PCBCL, the significance of the current staging approach as a risk profiling tool and the effect of various treatments on outcome remain unclear. We retrospectively reviewed patients who presented with a diagnosis of PCBCL seen at The Ohio State University between 1998 and 2012. We reviewed the initial presentation and treatment modality. We then assessed whether the treatment modality (conservative skin-directed vs. definitive radiation with or without systemic therapy), stage (T1 or ≥T2), or histologic subtype (primary cutaneous follicle center lymphoma [PCFCL] vs. primary cutaneous marginal zone B-cell lymphoma [PCMZL]) affected the risk of recurrence. We identified 67 patients referred with an initial diagnosis of PCBCL. After imaging, 12 did not meet the criteria for PCBCL and were classified as having systemic B-cell lymphoma with cutaneous involvement. The remaining 55 patients included 25 with PCMZL, 24 with PCFCL, 2 with primary cutaneous large B-cell lymphoma leg type, and 4 with unclassifiable disease. According to the International Society of Cutaneous Lymphoma-European Organization for Research and Treatment of Cancer staging, 30 cases were T1 (55%), 14 T2 (25%), and 11 T3 (20%). Comparing the time to first recurrence (TFR) by indolent PCBCL subtypes, we found no difference in the recurrence risk for either stage (T1, p = .51 vs. T2/T3, p = .30). Comparing TFR by treatment modality, we found no difference in TFR within T1 patients (p = .34) or T2/T3 patients (p = .44). Our limited analysis highlights the importance of complete staging at diagnosis and suggests that the treatment modality does not affect the risk of recurrence in T1 indolent PCBCL.

Molecular Characterization of Post-Transplant Plasmablastic Lymphomas Implicates RAS, TP53, and NOTCH Mutations and MYC Deregulation in Disease Pathogenesis

The diagnosis of diffuse large B-cell lymphoma (DLBCL) in the current era is complex and involves a subclassification scheme that incorporates morphologic, immunophenotypic, and genetic features. In addition, there are large B-cell lymphomas that have a unique biology or site predilection, which justifies the use of separate diagnostic terminologies (e.g., Epstein-Barr virus (EBV)+ DLBCL, not otherwise specified (NOS); primary DLBCL of the CNS; primary cutaneous DLBCL, leg type; DLBCL associated with chronic inflammation; plasmablastic lymphoma etc. ). This chapter covers large B-cell lymphomas that are known to involve the spleen, primary or secondary. The most common lymphoma of the spleen is DLBCL, NOS, and most of these are secondary in nature. We discuss both the common macronodular manifestation and the less common diffuse red pulp variant of DLBCLs. T-cell-/histiocyte-rich large B-cell lymphoma (TCRBL) has morphologic features (micronodular) unique to the spleen as compared to the typical morphology observed in nodal TCRBL. EBV+ DLBCL, NOS, of the spleen has morphology similar to that observed in other sites, however, with important differential diagnoses that need to be considered. Lastly, we discuss the recently introduced high-grade B-cell lymphomas (HGBL), which include double-/triple-hit lymphomas (involving MYC, BCL2, and/or BCL6 translocations) and high-grade B-cell lymphoma, NOS.

Double-hit or dual expression of MYC and BCL2 in primary cutaneous large B-cell lymphomas

Gastrointestinal Plasmoblastic Lymphoma Associated with Wiskott-Aldrich Syndrome: A Case Report

Poor Outcomes in HIV Negative and HIV Positive Patients with Plasmablastic Lymphoma (PBL) in a Population Based Patient Study

Plasmablastic lymphoma (PBL) is a rare variant of diffuse large B-cell lymphoma which has been most commonly reported in jaw and oral cavity in Human Immunodeficiency Virus (HIV) infected patients. A handful of cases have been reported in the extra-oral sites. Plasmablastic lymphomas should be differentiated from other large cell lymphomas as these lymphomas have very short survival and poor response to chemotherapy. We present a case of PBL in an HIV infected patient with history of recurrent anal fistulotomies.

Cutaneous Lymphomas — Part II: Other Cutaneous Lymphomas