Myeloablative Dose of Busulfan and Fludarabine Combined with In Vivo T Cell Depletion Is Safe and Effective Conditioning for Acute Myeloid Leukemia and Myelodysplastic Syndrome Patients.

Allogeneic hematopoietic stem cell transplantation (HSCT) is a curative strategy for acute myeloid leukemia (AML) and myelodysplastic syndromes (MDS). The prediction of transplantation-related mortality (TRM) using the Hematopoietic Cell Transplantation Comorbidity Index (HCT-CI) score and an arbitrary upper age limit of 55 years for administering myeloablative conditioning (MAC) are common strategies to ensure a safe procedure. The use of reduced-toxicity conditioning regimens is an additional approach to providing safe and effective myeloablation. Herein we report the outcome of AML and MDS patients conditioned with fludarabine and a myeloablative dose of busulfan (FB4) stratified by age and HCT-CI score. The primary objective was overall survival (OS) for patients age ≥55 years. Secondary objectives were total OS, TRM, graft-versus-host disease (GVHD), and GVHD, relapse-free survival (GRFS). The 2 year OS was 72% in patients age <55 and 51% in patients age ≥55. In patients age ≥55 with an HCT-CI <2, the estimated 2 year OS was 64%, with median OS not reached. In those with HCT-CI ≥2, the 2-year OS was 43%, with a median OS of 14 months. The total cumulative incidence of relapse was 30% regardless of age or HCT-CI score. FB4 conditioning regimen offers a high rate of prolonged remission with a relapse rate similar to that reported in previous studies. These positive outcomes suggest that this conditioning platform can be offered to patients age ≥55 years in the absence of comorbidities, and that age should not be the sole determinant of conditioning intensity.

Radioactive Iodine-Induced Chronic Myeloid Leukemia in a Patient With Graves' Disease: A Case Report.

Therapy-related leukemia is an increasing concern in hematology. One of these substances that showed to increase the incidence of leukemia is radioactive iodine (RAI). We report here a case of radioactive iodine-induced chronic myeloid leukemia (CML) in a patient with Graves' disease, although most cases in the literature were for thyroid cancer. Also, our patient received a very low dose, which is unique compared to previous case reports in the literature.

Severe hemolysis and vaso-occlusive crisis due to COVID-19 infection in a sickle cell disease patient improved after red blood cell exchange.

Even though most data suggest favorable outcome in patients with SCD and COVID-19 infection, close monitoring remains essential as acute complication may develop unexpectedly. Offering RBC exchange early in the course of infection might improve prognosis.

Highly Aggressive CD4-Positive Mast Cell Leukaemia (Leukaemic Variant) Associated with Isolated Trisomy 19 and Hemophagocytosis by Neoplastic Mast Cells: A Case Report with Challenging Experience and Review.

BACKGROUND: Mast cell leukaemia is a unique disease among hematopoietic neoplasms, being one of the rarest leukaemia subtypes. In addition, its prompt diagnosis is usually challenging. This is due to its heterogeneity in clinical presentations and cytomorphological and immunophenotypical features together with potential associations with other hematologic neoplasms which can complicate the condition and delay accurate diagnosis. To the best of our knowledge, this is the first case report of CD4-positive mast cell leukaemia. CASE PRESENTATION: A 39-year-old male presented with acute onset of fever, abdominal pain, and generalized body aches of two-week duration. Peripheral blood smear showed circulating blasts (13%) with coarsely basophilic granulation. Bone marrow (BM) aspirate showed extensive infiltration with immature mast cells of blast-like morphology with trilineage dysplasia and evident hemophagocytic activity exhibited by histiocytes and neoplastic mast cells. BM biopsy was diffusely infiltrated with many atypical mast cells positive for CD45, CD117, mast cell tryptase, CD25, and CD4 with partial positivity for CD7 and CD30. Cytogenetics showed an abnormal karyotype: 47, XY, +1947, XY, +19[13]/46, XY[9]. Molecular analysis revealed a KIT D816V mutation consistent with a diagnosis of systemic mastocytosis, mast cell leukaemia. CONCLUSION: The expression of T-cell associated markers by abnormal mast cells is well documented; however, CD4 and CD7 expression have not previously been described in association with mast cell leukaemia. Coexpression of CD2, CD4, CD7, and CD30 by the mast cells particularly in skin lesions may provoke misinterpretation as a cutaneous T-cell neoplasm. To the best of our knowledge, this is the first report of CD4-positive mast cell leukaemia. Moreover, hemophagocytic mast cell leukaemia is a very rare morphologic variant, and possible correlation between this finding and expression of CD4 by neoplastic mast cells is a topic for further investigation.

Risk Stratification of Necrotizing Fasciitis Based on the Initial Procalcitonin Concentration: A Single Center Observational Study.

BACKGROUND: Necrotizing fasciitis (NF) is a potentially fatal subcutaneous tissue and fascia infection. We studied the role of serum procalcitonin in the identification and assessment of severity of sepsis in patients with NF. METHODS: A retrospective analysis was conducted from January 2000 to December 2013 for all patients who admitted to surgical intensive care with provisional diagnosis of NF. Patients were categorized into four groups based on the initial procalcitonin concentrations (Group I: <0.5 low risk, Group II: ≥0.5-<2 moderate risk, Group III: ≥2-<10 high risk, and Group IV: ≥10 ng/mL high likelihood of severe sepsis). RESULTS: During the study period, 331 cases were identified to have NF with a mean age of 51 ± 14 years. Serum procalcitonin was tested in 62 cases (only between 2011 and December 2013) and all were positive (Group I: 22%, Group II: 18%, Group III: 21%, and Group IV: 39%). The most common affected regions were thigh and chest in Group II (46% and 9%, respectively), lower limbs in Group III (46%), and perineum and abdomen in Group IV (25% and 21%, respectively). In the four groups, 21 patients developed septic shock (Group I: 0%, Group II: 14%, Group III: 24%, and Group IV: 62%). The cut off procalcitonin value for septic shock was 5.6 ng/mL. Using receiver-operating characteristic curve, this cut off with the Area under the Curve (AUC) of 0.77 was found to have sensitivity 81% and specificity 67%. Sequential Organ Failure Assessment (SOFA) score was substantially greater in Group III and Group IV in comparison to Group I and Group II, p = 0.006. Procalcitonin levels were correlated well with SOFA score (r = 0.34, p = 0.007). There were 17 deaths in the four groups (Group I: 6%, Group II: 23%, Group III: 12%, and Group IV: 59%). CONCLUSION: Initial procalcitonin concentration in NF carries an important prognostic value and it correlates well with SOFA score and can predict the development of septic shock early in patients with NF.