Progression of a hepatosplenic gamma delta T-cell leukemia/lymphoma on hyperCVAD/MTX and ara-C: literature review and our institutional treatment approach

A 24-year-old male presented with abdominal pain, fever, and palpable splenomegaly. His differential count revealed myelocytes, metamyelocytes, and nucleated red cells. A bone marrow biopsy confirmed a diagnosis of hepatosplenic gamma delta T-cell leukemia/lymphoma. We describe here our center\u27s diagnostic and treatment approach for this rare leukemia

Taking abdominal pain seriously: a case of aggressive dedifferentiated liposarcoma

Case Description: A 64 year-old female with history of PE on Eliquis with IVC filter placement, tobacco dependence, and class III obesity presents with abdominal discomfort and bilateral leg swelling. Physical examination showed a distended abdomen and bilateral leg edema and tenderness. Doppler ultrasound of the lower extremities showed extensive bilateral lower extremity deep vein thromboses (DVT). Patient developed oliguria and acute kidney injury, and ultrasound of the kidney was obtained. Kidney U.S. showed two large masses in the abdomen and pelvis. CT of the abdomen/pelvis demonstrated a mesenteric mass measuring up to 26 cm in the right mid-abdomen with multiple adjacent smaller masses. Patient underwent IR biopsy of the mass which showed a malignant neoplasm, with core biopsies showing spindled and epithelioid cells. Molecular studies were positive for MDM2 amplification, with focal weak staining for CD10 and inhibin. These features were indicative of a dedifferentiated liposarcoma given presence of MDM2; however, a poorly differentiated sex cord stromal tumor was also possible given weak staining by inhibin and CD10. Patient was not a surgical candidate but was a candidate for systemic chemotherapy. She received treatment with one cycle of Adriamycin, Ifosfamide, and Mesna (AIM) which was complicated by Ifosfamide-induced encephalopathy. She then received palliative radiation therapy and unfortunately passed secondary to cardiac arrest from suspected pulmonary embolism. Discussion: Liposarcomas (LPS) are rare mesenchymal neoplasms involving the deep soft tissues. Dedifferentiated liposarcomas (DDLPS) are high-grade aggressive neoplasms typically occur in the retroperitoneum, and have six-fold the rate of local and metastatic recurrence and disease-specific mortality than that of well-differentiated liposarcomas (WDLPS). In patients with abdominal discomfort and bilateral extremity deep thromboses, a malignant process must be on the differential. DDLPS is a focal outgrowth from precursor WDLPS lesions; therefore, early detection of these tumors is key to prompt diagnosis and management.https://scholarlycommons.henryford.com/merf2020caserpt/1021/thumbnail.jp

Upper‐extremity deep venous thrombosis after whole blood donation: report of three cases from a single blood center

Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/111919/1/trf13034.pd

The long shadow of socioeconomic deprivation over the modern management of acute myeloid leukemia – time to unravel the challenges

Abstract Biological and non-biological variables unrelated to acute myeloid leukemia (AML) preclude standard therapy in many settings, with “real world” patients under-represented in clinical trials and prognostic models. Here, using a case-based format, we illustrate the impact that socioeconomic and anthropogeographical constraints can have on optimally managing AML in 4 different healthcare systems. The granular details provided, emphasize the need for the development and targeting of socioeconomic interventions that are commensurate with the changing landscape of AML therapeutics, in order to avoid worsening the disparity in outcomes between patients with biologically similar disease

ASSESSMENT OF OSTEOPOROSIS AND ANAEMIA RISK IN PATIENTS ON ANTICONVULSANT THERAPY

Objective: To assess the incidence of osteoporosis and anaemia in patients on anticonvulsant therapy and to educate those under risk.Methods: A prospective observational study was conducted on 50 study participants. The Bone mineral density (BMD), vitamin D, hematological parameters and peripheral smear were noted. Data analyzed using different statistical methods. Patient information brochures for osteoporosis and anaemia were distributed to those on chronic anti-epileptic drug (AED) therapy.Results: The prevalence of osteoporosis was 16% and osteopenia 22%. The BMD of subjects showed an Insignificant reduction in BMD when compared with a standard reference value for south asian population (*P>0.05). The mean BMD in single therapy group was higher compared with multiple therapy groups. BMD of the enzyme-inducing class was less compared with non-enzyme inducing class but was not significant (P>0.05). Duration of therapy was compared with BMD of patients showed a negative correlation. The relationship between duration of therapy and hematological parameters showed a negative correlation (r =-0.128). The mean haematological parameters in single AED therapy were higher when compared with multiple AED therapy. The study demonstrated 40% microcytic hypochromic and 4% macrocytic hypochromic morphology.Conclusion: Chronic therapy with AEDs possesses a significant risk of developing osteoporosis and anaemia. The incidence rate varies according to the type, duration, and mode of therapy. Early detection and management through diet exercise or pharmacotherapy will decrease the incidence of unwanted effects due to AEDs and improve the quality of life.Keywords: Bone mineral density, Antiepileptic drug, Osteoporosi

Impact of COVID-19 Infection on 24 Patients with Sickle Cell Disease One Center Urban Experience, Detroit, MI, USA

The city of Detroit has a large population of individuals with sickle cell disease, and hospitals in Detroit have seen some of the highest numbers of cases of coronavirus disease-19 (COVID-19) in 2020. The purpose of this study was to examine the pathophysiological characteristics of COVID-19 in patients with sickle cell disease or trait to determine whether these patients have unique manifestations that might require special consideration. This retrospective analysis included 24 patients with confirmed COVID-19 and sickle cell disease or trait who were seen at the Henry Ford Hospital, Detroit, MI, USA, between March 1 and April 15 2020. Of the 24 patients, 18 (75.0%) had heterozygous sickle cell trait, one (4.0%) was a double heterozygote for Hb S (HBB: c.20A\u3eT)/β(+)-thalassemia (β(+)-thal), four had sickle cell anemia (β(S)/β(S)) and one (4.0%) had Hb S/Hb C (HBB: c.19G\u3eA) disease. A total of 13 (54.0%) patients required hospitalization. All four patients with sickle cell anemia, developed acute pain crisis. We observed one patient who developed acute pulmonary embolism and no patients developed other sickle cell associated complications. Additionally, three (13.0%) patients required packed red blood cell transfusion without the need of exchange transfusion, and one patient required admission to the intensive care unit (ICU), mechanical ventilation and subsequently died. Patients with sickle cell disease or trait and laboratory-confirmed COVID-19 had a generally mild, or unremarkable, course of disease, with lower chances of intubation, ICU admission and death, but with a slightly longer hospitalization

The long shadow of socioeconomic deprivation over the modern management of acute myeloid leukemia: time to unravel the challenges

Biological and non-biological variables unrelated to acute myeloid leukemia (AML) preclude standard therapy in many settings, with “real world” patients under-represented in clinical trials and prognostic models. Here, using a case-based format, we illustrate the impact that socioeconomic and anthropogeographical constraints can have on optimally managing AML in 4 different healthcare systems. The granular details provided, emphasize the need for the development and targeting of socioeconomic interventions that are commensurate with the changing landscape of AML therapeutics, in order to avoid worsening the disparity in outcomes between patients with biologically similar disease

Sequential occurrence of thrombotic thrombocytopenic purpura, essential thrombocythemia, and idiopathic thrombocytopenic purpura in a 42-year-old African-American woman: a case report and review of the literature

<p>Abstract</p> <p>Introduction</p> <p>Thrombotic thrombocytopenic purpura and idiopathic thrombocytopenic purpura are two well recognized syndromes that are characterized by low platelet counts. In contrast, essential thrombocythemia is a myeloproliferative disease characterized by abnormally high platelet numbers.</p> <p>The coexistence of thrombotic thrombocytopenic purpura and idiopathic thrombocytopenic purpura in a single patient has been reported in the literature on a few occasions. However, having essential thrombocythemia complicating the picture has never been reported before.</p> <p>Case presentation</p> <p>We present a case where thrombotic thrombocytopenic purpura, essential thrombocythemia, and idiopathic thrombocytopenic purpura were diagnosed in a 42-year-old African-American woman in the space of a few years; we are reporting this case with the aim of drawing attention to this undocumented occurrence, which remains under investigation.</p> <p>Conclusions</p> <p>As the three conditions have different natural histories and require different treatment modalities, it is important to recognize that these diseases may be seen sequentially. This case emphasizes the importance of reviewing peripheral blood smears for evaluation of thrombocytopenia and bone marrow aspirations for diagnosis of thrombocythemia in order to reach an accurate diagnosis and tailor therapy accordingly. Moreover, this case demonstrates the variability and complexity of platelet disorders. This occurrence of three different types of platelet disorders in one patient remains a pure observation on our part; regardless, this does raise the possibility of a common underlying, as yet undiscovered, pathophysiology that could explain the phenomenon.</p

EFFICACY AND SAFETY OF EPTACOG BETA (RECOMBINANT HUMAN FVIIA) ACCORDING TO AGE IN PERSONS WITH HAEMOPHILIA A/B WITH INHIBITORS UNDERGOING SURGICAL PROCEDURES

Introduction: Eptacog beta (CEVENFACTA®) is a new rFVIIa approved by the EMA for the treatment of bleeding events and prevention of bleeding during surgery in persons with haemophilia A/B with inhibitors (PwHABI) aged ≥12 years (y). Methods: PERSEPT 3 was a Phase 3 (NCT02020369) trial of eptacog beta in PwHABI who required surgical procedures. Eptacog beta was administered at an initial dose of 200μg/kg or 75μg/kg for major or minor procedures respectively. This was followed by 75μg/kg for ≥5 (major procedures) or ≥2 (minor procedures) days. Haemostatic efficacy was assessed using a 4-point scale during the intra and postoperative care period (primary efficacy endpoint was determined by the investigator at the study centre 48±4h after the last dose of eptacog beta, based on the totality of the assessments performed on the patient (pt) at each timepoint). This post-hoc analysis compared the efficacy and safety of eptacog beta by age (pts aged \u3c12 vs ≥12y). Results: Twelve pts were included (\u3c12y: n=5, 1 major and 4 minor procedures; ≥12y: n=7, 5 major and 2 minor procedures). The primary endpoint success proportion was 100% (95% CI: 39.8-100) in pts aged \u3c12y (4 successes, 1 missing) and 71.4% (95% CI: 29.0-96.3) in pts aged ≥12y (5 successes; 2 failures). The intraoperative success proportion was 100% (95% CI: 47.8-100) for pts aged \u3c12y (5 successes) and 100% (95% CI: 59.0-100) for pts aged ≥12y (7 successes). The success proportion 24h post-procedure was 100% (95% CI: 47.8-100) for pts aged \u3c12y (5 successes) and 100% (95% CI: 47.8-100) for pts aged ≥12y (5 successes; 2 missing). Two pts discontinued treatment (1 aged \u3c12y withdrew consent; 1 aged ≥12y due to an adverse event (AE): postprocedural hematoma). One pt experienced 2 serious AEs leading to death, both were considered unrelated to the treatment. No allergic or thrombotic events occurred; no neutralising antibodies were detected. Antifibrinolytics were used concomitantly with eptacog beta in 4 patients without any safety concerns. Discussion/Conclusion: This post-hoc subgroup analysis shows that eptacog beta is effective and well-tolerated in perioperative care irrespective of patient age (\u3c12 vs ≥12y), supporting the use of eptacog beta for bleed management (prevention and treatment) in major and minor surgical procedures in all PwHABI