Myeloid/Lymphoid Neoplasm with FGFR1 Rearrangement Presenting with Polycythemia Vera and T-cell Acute Lymphoblastic Leukemia.

Myeloid/lymphoid neoplasm with fibroblast growth factor 1 rearrangements (MLN-FGFR1) represents a rare group of hematologic neoplasms, with approximately 100 cases reported to date. A 69-year-old woman with a history of polycythemia and leukocytosis, with negative molecular testing for JAK2, CALR, and MPL, presented with diffuse adenopathy. A lymph node (LN) biopsy revealed effacement by T-lymphoblasts, consistent with T-cell acute lymphoblastic lymphoma (T-ALL). A staging bone marrow (BM) biopsy demonstrated trilineage hyperplasia, which, taken together with the patient's elevated hemoglobin and low serum erythropoietin level, fulfilled diagnostic criteria for polycythemia vera. Karyotype and fluorescence in situ hybridization on both the BM and LN demonstrated a FGFR1 rearrangement due to t(8;13), consistent with MLN-FGFR1. Whole genome sequencing on the LN additionally identified a pathogenic frameshift mutation of ASXL1 NC_000020.11:g32434646dup NM_015338.6(ASXL1):c.1934dup p.(Gly646Trpfs) predicted to result in loss of protein function, a finding also observed in 8.1% of BM reads. Both the BM and LN harbored missense variants in HDAC4 NM_001378414.1(HDAC4):c.[2763G>A]; [2763=] p.(Met921Ile) and CHEK2 NM_007194.4(CHEK2):c.[538C>T];[538=] p.(Arg180Cys), with an unknown significance. Despite initial response to Mini-CVD + venetoclax, the patient subsequently experienced rapid clinical deterioration and death. We report the second case of MLN-FGFR1 with an ASXL1 mutation and the first case with HDAC4 and CHEK2 variants.

Survival of U.S. Military Service Members With Lymphoma.

INTRODUCTION: Cancer is a prominent cause of mortality in today's active duty service members (ADSMs), killing over 700 ADSMs between 2004 and 2015. Hence, more research is needed to better understand the survival of U.S. service members with cancer. Lymphoma is the most common hematologic malignancy diagnosed in ADSMs, serving as a good cancer type to study. MATERIALS AND METHODS: The Department of Defense tumor registry and the Surveillance, Epidemiology, and End Result (SEER) databases were retrospectively accessed to analyze overall survival (OS) of ADSMs with lymphoma and to compare outcomes with matched civilians. ADSMs with Hodgkin lymphoma (HL), diffuse large B-cell lymphoma (DLBCL), and indolent lymphoma diagnosed between 1997 and 2017 were included, and SEER comparator patients were matched by age, sex, race, stage, and year of diagnosis using a 4:1 ratio of civilians to ADSMs. RESULTS: There were 1,170, 443, and 284 ADSMs with HL, DLBCL, and indolent lymphoma, respectively, and all three groups had superior OS when compared to their matched civilian counterparts with 35%, 49%, and 48% reductions in the risk of death, respectively. There were no differences in ADSM survival based on race, sex, or rank, despite disparities being pervasive in the civilian sector. CONCLUSIONS: Service members with lymphoma have superior survival than their matched civilian counterparts, without evident racial or sex disparities. Results of this study are favorable in terms of readiness. Further research on cancer mortality in ADSMs is needed to improve long-term outcomes.

Clostridioides difficile-Associated Atypical Hemolytic-Uremic Syndrome Successfully Treated With Eculizumab: A Case Report and Literature Review.

Clostridioides difficile infection is a rare precipitant for patients to develop atypical hemolytic-uremic syndrome, of which the pathogenesis remains unclear. Previous reports suggest activation of cytokine storm from binding of cyotoxins A and B to colonic wall membranes. CASE SUMMARY: We present a case of a previously healthy 21-year-old woman who developed fulminant C. difficile colitis and atypical hemolytic-uremic syndrome requiring abdominal surgery and renal replacement therapy. She was ultimately treated with eculizumab without the use of plasmapheresis and remains in remission with full renal recovery. CONCLUSIONS: Our patient's significant response to terminal complement inhibitor, without the use of plasmapheresis, suggests that the underlying pathology is significantly driven by the alternative complement pathway. We propose that C. difficile-associated atypical hemolytic-uremic syndrome be defined as primary atypical hemolytic-uremic syndrome and strongly consider eculizumab as first-line therapy.

Colorectal Cancer in a Patient With Multiple Myeloma: A Treatment Dilemma.

Multiple myeloma (MM) is a malignancy of plasma cells characterized by the clonal proliferation of plasma cells that produce monoclonal immunoglobulins. While typically considered to be incurable, advances in treatment options have led to remarkable improvements in survival for these patients. Accumulating evidence suggests an increased risk for the development of a secondary primary malignancy (SPM) in these patients, perhaps as a result of myeloma directed therapy or as an effect of their underlying disease process. Here we present a case of a patient who was diagnosed with an SPM while undergoing palliative treatment for multiple myeloma and a treatment approach.

Chronic Lymphocytic Leukemia as an Unusual Cause of Rapid Airway Compromise.

Chronic Lymphocytic Leukemia (CLL) is the most prevalent form of non-Hodgkin's lymphoma (NHL) in Western countries predominantly affecting adults over the age of 65. CLL is commonly indolent in nature but can present locally and aggressively at extranodal sites. Although CLL may commonly present with cervical lymphadenopathy, manifestation in nonlymphoid regions of the head and neck is not well described. CLL causing upper airway obstruction is even more uncommon. We describe a case of a patient with known history of CLL and stable lymphocytosis that developed an enlarging lymphoid base of tongue (BOT) mass resulting in rapid airway compromise.