Remarkable Response of Hairy Cell Leukemia Variant to Single-Agent Cladribine.

Classical hairy cell leukemia (cHCL) and related mature lymphoid B-cell neoplasms including hairy cell leukemia variant (HCLv) and splenic diffuse red pulp lymphoma (SDRPL) are a rare subset of lymphoid neoplasms. cHCL accounts for around 2% of all leukemias and is characterized by a peripheral smear with large lymphoid cells with cytoplasmic projections giving the cells a hairy appearance, splenomegaly, and cytopenias. Majority of cHCL cases harbor a BRAFV600E mutation. cHCL usually responds well to single-agent purine analogs. HCLv is even rarer and constitutes around 0.4% of lymphoid malignancies. Unlike cHCL, HCLv is less responsive to standard single-agent purine analogs and typically does not harbor the BRAFV600E mutation. The "hairy cells," splenomegaly, and cytopenias are common in both. We report a case of a patient with HCLv who was treated with a single purine analog and achieved a near-complete response.

Managing Massive Splenic Hematoma: Exploratory Laparotomy or Active Surveillance?

Splenic hematoma is a known complication of blunt force abdominal trauma. Traditional management of splenic hematomas has been primarily surgical. However, more recently, spleen-sparing management has been favored over surgical management for cases that meet certain criteria, with surgery now reserved for patients with complications. In this report, we present a case of a massive splenic hematoma that was managed conservatively and analyze the challenges faced in clinical decision making.

Tackling Diversity in Prostate Cancer Clinical Trials: A Report From the Diversity Working Group of the IRONMAN Registry.

Prostate cancer disproportionately affects racial and ethnic minority populations. Reasons for disparate outcomes among minority patients are multifaceted and complex, involving factors at the patient, provider, and system levels. Although advancements in our understanding of disease biology have led to novel therapeutics for men with advanced prostate cancer, including the introduction of biomarker-driven therapeutics, pivotal translational studies and clinical trials are underrepresented by minority populations. Despite attempts to bridge the disparities gap, there remains an unmet need to expand minority engagement and participation in clinical trials to better define the impact of therapy on efficacy outcomes, quality of life, and role of biomarkers in diverse patient populations. The IRONMAN registry (ClinicalTrials.gov identifier: NCT03151629), a global, prospective, population-based study, was borne from this unmet medical need to address persistent gaps in our knowledge of advanced prostate cancer. Through integrated collection of clinical outcomes, patient-reported outcomes, epidemiologic data, and biospecimens, IRONMAN has the goal of expanding our understanding of how and why prostate cancer outcomes differ by race and ethnicity. To this end, the Diversity Working Group of the IRONMAN registry has developed informed strategies for site selection, recruitment, engagement and retention, and trial design and eligibility criteria to ensure broad inclusion and needs awareness of minority participants. In concert with systematic strategies to tackle the complex levels of disparate care, our ultimate goal is to expand minority engagement in clinical research and bridge the disparities gap in prostate cancer care.

The Pulmonary Complications of Paraneoplastic Autoimmune Vasculitis in a Patient With Myelodysplastic Syndrome.

Paraneoplastic autoimmune phenomena may occur in up to 30% of patients with myelodysplastic syndrome (MDS). We present the case of a patient with MDS who developed diffuse alveolar hemorrhage due to paraneoplastic autoimmune vasculitis. The patient was a 55-year-old male who had been referred for outpatient hematology/oncology evaluation by his primary care physician for incidentally discovered thrombocytopenia. When he presented to the clinic, he reported new-onset chills, weakness, and night sweats. He endorsed a 20-pound weight loss over two months as well as two weeks of fatigue, exertional dyspnea, and epistaxis. He was noted to be ill-appearing and had bilateral pitting edema to the knees. Vital signs revealed a temperature of 102.3 °F, oxygen saturation of 84% on room air, and tachycardia to the 90s. Labs showed hemoglobin of 5.7 g/dL, hematocrit of 17.2 g/dL, and platelet count of 27 kµL. He was admitted to the hospital for blood and platelet transfusions, empiric antibiotics, and further diagnostic studies. The peripheral blood smear showed 4% blasts and frequent dyspoietic granulocytes. Bone marrow biopsy (BMB) was performed to differentiate between acute leukemia and myelodysplasia. BMB revealed myelodysplasia with excess blasts and erythroid predominance.During hospitalization, the patient developed acute hypoxemic respiratory failure due to bronchoscopy-confirmed diffuse alveolar hemorrhage from thrombocytopenia. His platelet count was 12 kµL. High-dose corticosteroids (2 mg/kg prednisone) were initiated for suspected paraneoplastic autoimmune vasculitis, pending BMB results. The patient steadily improved, was extubated, and had reduced oxygen and transfusion requirements.High-dose steroids were stopped, and the patient was started on decitabine chemotherapy with the ultimate goal of bone marrow transplantation. On day five of decitabine, the patient developed acute hypoxic respiratory failure requiring intubation as well as hypotension requiring vasopressors. Given that recurrent diffuse alveolar hemorrhage was again suspected, high-dose steroids were resumed upon transfer to the ICU. He continued to decompensate and ultimately experienced ventricular tachycardia requiring three separate episodes of cardiopulmonary resuscitation. Per the family's wishes, he was palliatively extubated, and he expired an hour later. Diffuse alveolar hemorrhage is a rare but potentially deadly pulmonary complication of MDS, stemming from a paraneoplastic autoimmune vasculitis. Patients who initially present with atypical autoimmune phenomena should raise suspicion for an underlying MDS, the presence of which can guide the promptness, extent, and duration of immunosuppressive therapy. Failure to expeditiously treat these patients with corticosteroids can lead to serious complications and death.