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We describe the prognostic implication and aggressive clinical course of lymphoma-related lactic acidosis in a rare HIV-related lymphoma. Patient was diagnosed with plasmablastic lymphoma and developed severe lactic acidosis, and was treated on the medical floor and in the medical intensive care unit. Her lactic acidosis was considered to be type B, secondary to her underlying lymphoma since she never had an infectious source, hypovolemic state, or low/high cardiac-output state. The mechanism of the lymphoma-related lactic acidosis is from altered cellular metabolism, thought to aid in lymphoma proliferation, rather than tissue hypoperfusion. It is a rare complication of aggressive lymphomas and signifies a poor prognosis. Patients having this complication should be considered for close monitoring and management in an intensive care unit until definitive treatment (i.e., chemotherapy) can be implemented.

Hypercalcemia in malignancy is associated with multiple mechanisms and occurs in up to 20–30% of cancer patients. We report a case of small lymphocytic lymphoma/chronic lymphocytic leukemia (SLL/CLL) associated with hypercalcemia and an elevation in parathyroid hormone-related peptide (PTHrP) in the setting of a Richter transformation. Real-time reverse transcriptase PCR on lymph node biopsy specimens obtained before and after transformation showed an 8-fold increase in PTHrP mRNA levels and about 2-fold decrease in the levels of its cognate receptor PTHR1. The findings of this case suggest that parathyroid hormone-related peptide might be useful in monitoring a specific group of patients with SLL/CLL who develop hypercalcemia during the course of their disease and could suggest an autocrine-like mechanism involving PTHrP in Richter transformation.

Background Large clonal populations of cells bearing PIG - A mutations are the sine qua non of PNH, but the PIG - A mutation itself is insufficient for clonal expansion. The association between PNH and aplastic anemia supports the immune escape model, but not all PNH patients demonstrate a history of aplasia; therefore, second genetic hits driving clonal expansion have been postulated. Based on the previous identification of JAK2 mutations in patients with a myeloproliferative/PNH overlap syndrome, we considered TET2 as a candidate gene in which mutations might be contributing to clonal expansion. Methods Here we sequenced the TET2 and JAK2 genes in 19 patients with large PNH clones. Results We found one patient with a novel somatic nonsense mutation in TET2 in multiple hematopoietic lineages, which was detectable upon repeat testing. This patient has had severe thromboses and has relatively higher peripheral blood counts compared with the other patients—but does not have other features of a myeloproliferative neoplasm. Conclusions We conclude that mutations in TET2 may contribute to clonal expansion in exceptional cases of PNH.

Abstract Primary dura mater–based lymphomas are extremely uncommon and when detected are often clinically and radiologically misidentified. As per literature search, they account for <0.1% of all non-Hodgkin lymphomas; however, a precise incidence is unknown since only a few cases have been described in the literature. Here we report a case of a 64-year-old female who presented for evaluation of a newly diagnosed left tentorial tumor, diagnosed as “consistent with meningioma” on imaging studies with significant mass effect on the left occipital lobe and left cerebellum with effacement of the fourth ventricle. She had been experiencing disabling headaches, balance dysfunction, and reduced vision. On examination, the patient had right visual field defect with wide-based gait. No evidence of systemic lymphoma or clinically suspicious lymphadenopathy was documented. An intraoperative consultation revealed sheets of lymphocytes, rather highly concerning for lymphoma. H&E sections showed dense fibroconnective tissue heavily infiltrated by mature small- to intermediate-sized lymphocytes with irregular nuclei and condensed chromatin without a discernible architecture. Only sparse and scattered larger lymphocytes were seen. Flow cytometry identified a subset of B cells with lambda-restricted immunophenotype expressing CD19+, CD20+, CD10+, CD5–, and CD23–. On tissue sections, Ki-67 showed an overall proliferation index of 10% to 20%. However, no residual follicular dendritic cell meshwork was detected by CD21 or CD23. PCR analysis detected clonal B-cell IgH and IgKappa gene rearrangements. A diagnosis of low-grade CD10-positive B-cell lymphoma (likely follicle center-cell origin) was made and the patient was discharged after 3 days of surgery for outpatient follow-up and treatment. In conclusion, low-grade dural-based lymphomas are extremely rare and often misdiagnosed as meningiomas clinically and radiologically. Additionally, it is important distinguish lymphomas of the dura mater, which are excluded from the definition of primary CNS lymphomas and may have a different clinical management and outcome.

Primary mediastinal large B-cell lymphoma (PMBCL) is a rare type of non-Hodgkin lymphoma that typically has a good response rate to first line chemotherapy regimens. There have been reports of successful chemotherapy, but with a residual mass from fibrosis. Here, we report the case of a 19-year-old primigravida presenting with cardiogenic shock and superior vena cava (SVC) syndrome at full term who was found to have a PMBCL. Following delivery via urgent cesarean section, she was put on veno-arterial extra corporeal membrane oxygenation (VA-ECMO) and once hemodynamically stable was started on chemotherapy. In view of limited change in tumor size on consecutive CT scans and questionable response to chemotherapy, there were multidisciplinary meetings wherein withdrawing support was discussed and put forward to the family. At that point, surgical debulking was offered on compassionate grounds to be able to wean her off the VA-ECMO. This case report highlights the role of salvage resection when there are no other options.

: The gastrointestinal tract is the most common extranodal site for non-Hodgkin's lymphoma, with the most common being diffuse large B cell lymphoma. Unlike the stomach or the ileum, the jejunum is a rare site for primary extranodal lymphomas, given the scarcity of lymphoid tissue. Due to its location, inflammation in the jejunum may not be visualized on routine imaging or endoscopy, making jejunal lymphoma difficult to diagnose. : We present a case of a 90-year-old male with 1 week of intractable emesis, initially thought to be due to viral gastroenteritis. His symptoms never improved and he underwent serial CT imagings in addition to esophagogastroduodenoscopy. A stomach biopsy and a diagnostic paracentesis did not reveal any malignant cells, but a CT enterography revealed significant jejunal inflammation with obstruction. After a month of hospitalization, a jejunal biopsy was obtained, which showed proliferation of neoplastic B cells. He was ultimately diagnosed with primary jejunal diffuse large B cell lymphoma. : Chemotherapy and surgical resection are typically the definitive treatment for extranodal lymphoma. Clinicians, however, must carefully consider the patient's functional and nutritional statuses before offering such interventions. This case was a diagnostic challenge and demonstrated a rare GI malignancy's convoluted mimicking nature.

T-cell lineage lymphoma with an intense membranous and paranuclear CD30 expression in the absence of ALK1 raises a differential diagnosis of peripheral T-cell lymphoma (PTCL), NOS and anaplastic large cell lymphoma (ALCL), ALK negative. However, Epstein-Barr virus is consistently negative in ALCL and is not considered an implicating factor in its pathogenesis. We describe a case of T-cell lymphoma showing anaplastic large cell morphology with scattered hallmark cells and a uniform CD30 and Epstein-Barr virus encoded early RNA (EBER) expression that primarily involved the subcutaneous tissue at presentation. On incisional biopsy, the neoplastic cells were positive for CD3, CD2, and CD30 while negative for LCA, CD20, PAX5, CD56, ALK1, and cytotoxic granules. Molecular analysis identified a positive T-cell receptor (beta and gamma) gene rearrangement by PCR. Proliferation index approached 100% and the patient had a rapidly progressive course; the subcutaneous lesions more than doubled in size within couple of weeks with new evidence for widespread systemic involvement. This case emphasizes a rare EBV association with a CD30 positive T-cell lymphoma where the morphologic and immunophenotypic findings are otherwise nondiscriminatory between PTCL, NOS and ALCL, ALK negative.

We reviewed FLT3 and NPM1 mutation data in a large cohort of patients with myelodysplastic syndrome (MDS). The frequencies of FLT3 and NPM1 mutation were 2.0% and 4.4%, respectively, and mutations were restricted to cases of intermediate- and high-risk MDS. Cytogenetic abnormalities were identified in 46.9% of cases. FLT3 mutations were associated with a complex karyotype (P = .009), whereas NPM1 mutations were associated with a diploid karyotype (P < .001). FLT3 mutation (P < .001) was associated with progression to acute myeloid leukemia (AML), as were a higher bone marrow (BM) blast count (P < .001) and complex cytogenetics (P = .039). No patient with an NPM1 mutation alone had disease that progressed to AML. Cox proportional regression multivariate analysis indicated that FLT3 mutation, NPM1 mutation, complex cytogenetics, BM blast count, pancytopenia, and age were independent factors that correlated with progression-free survival. We conclude that FLT3 and NPM1 mutations are rare in MDS, but assessment of mutation status is potentially useful for predicting progression to AML.