Delineation of clinical course, outcomes, and prognostic factors in patients with T-cell prolymphocytic leukemia.

T-cell prolymphocytic leukemia (T-PLL) is a rare, post-thymic T-cell neoplasm with a diverse clinical course. T-PLL is typically associated with a poor prognosis; however, a subset of patients have inactive disease on initial presentation. There is a lack of accurate delineation of the disease based on initial clinical presentation and pathological assessment, hindering clinical decision-making. To characterize and delineate disease subtypes based on initial clinical presentation and pathologic assessment, we retrospectively reviewed 81 patients with T-PLL treated at our institution. We compared patients with T-PLL who initially presented with a relatively indolent or stable disease course to those with an aggressive disease course. Clinicopathologic characteristics, overall survival (OS), and prognostic factors were analyzed. Patients with inactive disease had a significantly longer OS than patients with active disease. At diagnosis, presence of B symptoms, low hemoglobin, low platelet count, lymphocyte doubling time of fewer than 3 months, and abnormal cytogenetics were associated with shorter OS. Cell morphology, immunophenotype, absolute lymphocyte count, lactate dehydrogenase levels, involvement of liver, spleen, skin or central nervous system, presence of TCL1 rearrangement or inv (14)/t(14;14), presence of chromosome 8 abnormalities, and presence of deletion of 11q were not associated with significant OS difference among the patients. Receiving alemtuzumab as first-line treatment and consolidation with allogeneic hematopoietic stem cell transplant were associated with better outcomes. T-PLL inactive and active disease subtypes can exhibit overlapping yet different clinical and pathological features. We describe several prognostic factors at diagnosis that can be used for risk stratification and aid in guiding treatment decisions.

An Unusual Presentation of Cryoglobulinemia in a Patient With Undiagnosed Sjögren's Syndrome and Treated Marginal Zone Lymphoma.

Our case details a 47-year-old female who presented to our cancer hospital with a petechial rash of the lower extremities as well as a headache and blurred vision for the prior two days. She was found to have systolic pressures in the 200s in the emergency department and was admitted for a hypertensive emergency. Notable medical history includes marginal zone lymphoma (MZL) status post-submandibular resection at an outside institution in 11/2017 (thought to be in remission). With her history in mind, she also reported subjective submandibular swelling on admission and an unintentional 25-pound weight loss over the eight weeks prior to admission. A PET scan was completed, which showed diffusely increased reticuloendothelial activity, and a follow-up bone marrow biopsy was without residual lymphoma activity. Creatinine was markedly elevated with significant proteinuria, and a renal biopsy revealed thrombotic microangiopathy, acute tubular injury, and moderate interstitial fibrosis. Remarkable laboratory tests included positive quantitative cryoglobulins ("cryocrit") and low complement 4 (C4). Qualitative cryoglobulins were never obtained, unfortunately. She was started on prednisone and transferred to a nearby academic hospital for formal rheumatologic evaluation. Importantly, testing at this facility showed elevated Sjögren's syndrome-related antigen A (SSA/Ro) antibodies. Also elicited at the academic hospital was that she had been experiencing symptoms of chronic dry eyes and mouth years even before her diagnosis of MZL. She was diagnosed with primary Sjögren's syndrome, which was thought to be the cause of her likely mixed cryoglobulinemia and the precipitant of her acute renal failure with hypertensive emergency, her skin changes, her anemia, and her hypocomplementemia. Of note, prior to discharge from the academic hospital, the patient's cryoglobulin testing was negative after prolonged steroid treatment, and she was placed on rituximab for maintenance. Our case is important as it helps illustrate one of the myriad precipitants of mixed cryoglobulinemia, in this case possibly untreated Sjögren's syndrome.

SARS-CoV-2 Infection (COVID-19) and Herpes Simplex Virus-1 Conjunctivitis: Concurrent Viral Infections or a Cause-Effect Result?

The pulmonary effects of severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2), the virus that causes coronavirus disease (COVID-19), are well documented; however, more evidence is needed to understand its effect on multiple organ systems. We present the case of a 69-year-old male with dyspnea for two weeks and bilateral conjunctivitis who tested positive for SARS-CoV-2. He was found to be hypoxic, requiring supplemental oxygen. On hospital day two, he complained of worsening left eye pain with the development of a left lower eyelid ulcer. He underwent a CT of facial bones, which showed findings consistent with pre-septal cellulitis and abscess. Samples from bilateral conjunctival secretions and left lower eyelid ulcer tested positive for herpes simplex virus-1 (HSV-1), and negative for SARS-CoV-2. He received supportive care, antibiotics, and famciclovir with almost complete resolution of his ocular complaints. This case illustrates an atypical COVID-19 presentation and raises concern as to how this virus modulates the immune system, allowing for concurrent viral infections.

Correlation of Venous Clinical Severity Score With Dermatology Life Quality Index Among Patients With Chronic Venous Insufficiency: A Cross-Sectional Study.

Introduction Chronic venous insufficiency (CVI) is characterized by inadequate functioning of venous valves in the lower limb. CVI is associated with a significant reduction in patient's quality of life (QOL). The severity of CVI was determined by CEAP (clinical, etiological, anatomical, pathophysiological) classification and venous clinical severity score (VCSS). The study is aimed to evaluate and correlate Dermatology Life Quality Index (DLQI) with VCSS, CEAP in patients with CVI. Methods A cross-sectional study of 57 patients with CVI was conducted over a period of 12 months. A sociographic survey, clinical and severity grading using CEAP classification, and VCSS were done for all venous doppler confirmed patients. QOL was evaluated by validated DLQI questionnaires using English and native languages Hindi and Marathi. Results A total of 57 patients with a male to female ratio of 6.1:1 and a mean age of 51.68 years were included in the study. CEAP grading in patients showed 49.12% (C4a), 21.05% (C6), 15.7% (C4b), 7.01% (C3), 3.50% (C2 and C5). Mean VCSS and DLQI were 11.47 and 10.12, respectively; 49.12%, 40.35%, 10.53% of patients had a moderate, very large, and small impact on DLQI respectively, positively correlating to VCSS (P < 0.001). Conclusion From this study, it was observed that VCSS and CEAP positively correlated with DLQI, and the impact increases in proportion with the seriousness of the disease.

MYC overexpression is associated with an early disease progression from MDS to AML.

Recent studies demonstrated that MYC epigenetically regulates AML cell survival and differentiation by suppressing IDH1/2-TET2-5hmC signaling and that MYC overexpression is associated with poor survival outcomes in multiple AML patient cohorts. However, the oncogenic roles of MYC in MDS remain to be explored. A total of 41 patients with de novo MDS were retrospectively identified using the Total Cancer Care database at the Moffitt Cancer Center. A total of 61 % of patients had low MYC expression and 39 % of patients had high MYC expression defined as MYC reactivity by immunohistochemical staining in ≥5% of bone marrow (BM) cells at the time of MDS diagnosis. The median MDS-to-AML progression free survival (PFS) was significantly shorter in the high MYC group (median PFS 9.3 vs. 17.7 months, HR = 2.328, p = 0.013). Further, overall survival (OS) was also shorter in the high MYC patients (median OS 19.7 vs. 51.7 months, HR = 2.299, p = 0.053). Multivariate analyses demonstrated that high MYC expression is an independent poor prognostic factor for the MDS-to-AML progression (HR = 2.275, p = 0.046). Our observations indicate that MYC may play a crucial role in MDS transformation to AML and the underlying mechanisms of MYC-driven MDS clonal expansion and leukemic transformation require further investigation.