A Rare Case of Ovarian Double-Hit/Diffuse Large B-Cell Lymphoma: A Case Report and Review of Literature.

Primary ovarian non-Hodgkin lymphoma is a rare lymphoma that is often associated with diagnostic delays, initial misdiagnosis, and inappropriate management. We report a case of ovarian diffuse large B-cell lymphoma (DLBCL) in a young female who initially presented with generalized fatigue, lower abdominal discomfort, and 40 pounds of unintentional weight loss. She subsequently had a computed tomography of abdomen done that showed fatty liver, hepatomegaly, and a left heterogeneous ovarian mass measuring about 4 × 4.2 cm. Transvaginal ultrasound was also done that showed a heterogeneous solid left adnexal mass measuring 7.4 × 5.6 × 6.6 cm. She subsequently had a total abdominal hysterectomy with bilateral salpingo-oophorectomy. Immunohistochemistry (IHC) showed the malignant cells expressing PAX5, CD20, and BCL2 with a Ki-67 proliferation index greater than 90%. The cells were negative for AE1/AE3, S100, CD30, and cyclin D1. Aggressive B-cell lymphoma fluorescence in situ hybridisation (FISH) panel was positive for rearrangement of BCL6 and MYC, with no evidence of BCL2 rearrangement, consistent with a double-hit high-grade B-cell lymphoma. Immunohistochemistry for BCL6 and MU M1 showed positive staining in the malignant cells. CD10 was negative. The staining profile was consistent with nongerminal center B-cell-like type of DLBCL. Ovarian lymphoma is a very rare entity; the presence of an enlarged ovarian tumor should raise the suspicion of ovarian lymphoma, and our case also emphasizes on the use of IHC markers in diagnosing the ovarian DLBCL.

Nodular Lymphocyte-Predominant Hodgkin Lymphoma: Review of Current Literature and Case Discussion.

Nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) is a rare subset of Hodgkin lymphoma (HL). It has a distinct clinical and pathological presentation. Unlike classic HL, where the predominant malignant cells are Reed Sternberg cells, the malignant cells in NLPHL are known as lymphocyte predominant (LP) cells, with their own unique immunohistochemistry antigen expression and staining pattern. Based on risk stratification and staging of the disease, treatment can range from active surveillance in asymptomatic patients with no organ compromise or bulky disease, to aggressive chemotherapeutic agents in advanced disease. Guidelines on which of these chemotherapy regimens would offer the most benefit to our patients are limited due to lack of randomized-controlled studies. Majority of the current prospective data on treatment were inclusive of both HL and NLPHL. Thus, the regimens employed in treatment of NLPHL are similar to the ones used in HL, though NLPHL is often viewed as its own distinct entity. This article aims to review the current literature and future advances on treatment of this rare disease.

Paraneoplastic Evans Syndrome in a Patient With Prostate Cancer With Small Cell Transformation.

Paraneoplastic syndromes are defined as tumor-associated indirect systemic effects. Prostate cancer-associated paraneoplastic syndromes typically have endocrine, neurologic, and dermatologic manifestations. Reports have suggested up to 70% of metastatic prostate cancers manifest as paraneoplastic entities. Although common in hematological malignancies, it is rare for prostate cancer and other solid tumors to be associated with immune-mediated cytopenias such as Evans syndrome. Based on our PubMed search for the keywords Evans syndrome and prostate cancer, only one other case has been reported in the literature with this association. We report the second such case in a 63-year-old male who initially presented with hemolytic anemia and thrombocytopenia. He was diagnosed with Evans syndrome with initial responses to both steroids and intravenous immunoglobulin. Extensive workup, including an eventual bone marrow biopsy, revealed metastatic prostate cancer with transformation to small cell neuroendocrine carcinoma.

DNA Methyl Transferase 3A (DNMT3A) Mutation Presenting as Isolated Pure Red Cell Aplasia.

Pure red cell aplasia (PRCA) is a rare disorder mainly affecting the erythroid precursor cells. It presents with severe isolated reticulocytopenia with relatively normal counts in the myeloid and megakaryocytic lineages. It has been attributed to numerous congenital and acquired causes. DNA Methyl Transferase 3 Alpha (DNMT3A) mutation has been typically associated with myeloid and lymphoid malignancies. There is a scarcity of data regarding the association of DNMT3A mutation with PRCA. We report a case of a 73-year-old man who initially presented with anemia and reticulocytopenia. After a thorough evaluation and eventual bone marrow biopsy, he was diagnosed with PRCA. Further genetic testing identified a DNMT3A mutation. We are reporting this rare case to highlight the fact that DNMT3A mutation can also present as isolated PRCA in and of itself without the co-occurrence of leukemia, lymphoma, or myelodysplastic syndrome (MDS).

Waldenstrom Macroglobulinemia Manifesting as Acute Kidney Injury and Bing-Neel Syndrome With Excellent Response to Ibrutinib.

Waldenstrom macroglobulinemia (WM) is a lymphoplasmacytic lymphoma associated with a monoclonal immunoglobulin M protein. Extranodal involvement in WM is not very common. In this article, we present a rare case of WM with kidney and central nervous system involvement. Bing-Neel syndrome is a distinct complication of WM where lymphoplasmacytic cells involve the central nervous system (CNS). Our patient was initially treated with dialysis and steroids with improvement in his kidney function. He was then started on systemic treatment with rituximab, cyclophosphamide, and dexamethasone with stable kidney function but persistent CNS symptoms. Due to rarity of cases, there is no standard treatment for Bing-Neel syndrome. His treatment was switched to ibrutinib with dramatic improvement in his CNS symptoms as well as radiological findings on magnetic resonance imaging.