Successful Treatment of Pure Red Cell Aplasia and Chronic GVH with Rituximab after ABO Mismatched HSCT

Background: Pure Red Cell Aplasia (PRCA) is a rare complication of ABO mismatched hematopoietic stem cell transplantation; there isn’t no standard of care, here we report a case of successful treatment by Rituximab in a refractory PRCA and chronic graft versus host disease. Case Presentation: A 26-year-old woman with PRCA following ABOmismatched allogeneic HSCT for chronic myeloid leukemia, associated with steroid refractory chronic hepatic graft versus host disease, treated with 4 doses of Rituximab 375mg/m² weekly, with an increase in her hemoglobin level and improvement of her liver’s enzymes. Conclusion: The interest of this case is to report the important therapeutic result of Rituximab, widely used in literature, especially if chronic Graft Versus host disease is associated.

Immune Reconstitution Inflammatory Syndrome Associated with Hepatosplenic Candidiasis in a Patient with Acute Myeloblastic Leukemia: Possible Pathogenesis and Treatment in the Light of Current Knowledge

Acquired immune deficiencies caused by different etiologies, promote invasive fungal infections. When this immunity begins to improve, it can induce an excessive inflammatory response defined as Immune Reconstitution Inflammatory Syndrome (IRIS). Hepatosplenic Candidiasis (HSC) can be considered a form of IRIS syndrome as it occurs following neutrophil recovery in patients treated for acute leukemia. Differentiating IRIS from a single fungal infection or treatment failure due to a similar clinical picture is a real diagnostic problem. Misdiagnosis and subsequently ineffective treatment with antifungal therapy instead of anti-inflammatory drugs, may lead fatal course of the disease. A deep and prolonged neutropenia developed after the first induction chemotherapy in our two and a half-year-old male patient who was followed up in our clinic with the diagnosis of Acute Myeloblastic Leukemia (AML). Our patient had fever, abdominal pain as well as his Gamma Glutamyl Transferase (GGT) and Alkaline Phosphatase (ALP) levels increased during neutropenia recovery. He was diagnosed with hepatosplenic candidiasis, by observing ‘target like abscesses’ on dynamic Magnetic Resonance Imaging (MRI) taken for his newly developing symptoms and laboratory findings while recovering neutropenia. After his first and third induction chemotherapy courses, his fever persisted although antifungal therapy, steroid treatment was initiated considering IRIS. After his re-intensification course, because of the same flare-up symptoms, we started immunglobulin in addition to steroid. With methylprednisolone and intravenous immunoglobulin, his symptoms improved and significant regression was observed in the lesions ‘target-like abscesses’ on MRI and in the laboratory values. Result: IRIS should be considered for patients with hepatic candidiasis whose have persistent fever despite appropriate antifungal therapy. Glucocorticoid should be started first for an anti-inflammatory effect.

Transfusion Related Acute Lung Injury (TRALI): A Rare Case after Single Packed Red Blood Cell (PRBC) Unit Transfusion

Transfusion Related Acute Lung Injury (TRALI) is a rare but serious adverse event of allogeneic blood component transfusion, manifested typically by shortness of breath, a non-productive cough, fever, and hypotension, mostly seen after plasma component transfusion collected from female donors. We here present a rare case of TRALI requiring Intensive Care Unit (ICU) support after transfusion of single Packed Red Blood Cell (PRBC) unit collected from a male donor. The present case emphasizes that TRALI to be ruled out first in any patient showing acute /respiratory distress within 6hrs of transfusion, with prompt management and notification to transfusion services.

Tricuspid Annular Plane Systolic Excursion as a Potential Marker of Hospital Utilization in Patients with Sickle Cell Disease

Objective: Pulmonary Artery Hypertension (PAH) is an independent risk factor for increased morbidity in Sickle Cell Disease (SCD) patients. Tricuspid Annular Plane Systolic Excursion (TAPSE) can be used as a surrogate measure for PAH. The objective of this study was to determine whether lower TAPSE values are associated with increased Emergency Department (ED) and hospital utilization. Methods: TAPSE measurements were retrospectively obtained from bedside echocardiograms from a convenience sample of 28 SCD patients presenting to the ED with SCD pain. TAPSE was considered abnormal if <24.9mm, one standard deviation below the mean TAPSE for SCD patients. Medical records were reviewed to determine ED visits and hospital overnight stays over a two-year period. A t-test analysis and Pearson’s correlation were used for each variable. Results: The initial sample included 28 SCD patient encounters. TAPSE measurements were abnormal in 5 patients and normal in 23 patients. The mean number of ED visits/year for the abnormal and normal TAPSE group were 23.00 and 16.87, respectively (p=0.57) with moderate negative linearity (p=0.03). The mean number of hospitalized days for abnormal and normal TAPSE groups was 108.8 and 59.6, respectively (p=0.10) with moderate negative linearity (p=0.07). Conclusion: Lower TAPSE values (<24.9mm) in SCD patients were associated with higher ED and hospital utilization. If findings are replicated in larger studies, TAPSE may serve as a marker of morbidity in SCD patients presenting to the ED.

Retrospective Study of Blood Transfusion Complications in the Capital Region of Denmark from 1999-2017: Characteristics of Potentially “Dangerous” Blood Donors?s

Objectives: We hypothesized that the blood donors most frequently involved in complications would induce more and severe immunologic transfusion complications compared to other donors, i.e. potentially “dangerous”. Secondary aims were differences in demographic variables. Background: Donor-related mechanisms may contribute to allogeneic blood transfusion complications and may represent a dangerous treatment adverse event. Materials and Methods: By analyzing transfusion data from the Capital Region of Denmark from January 1, 1999 to December 31, 2017; 2,574,646 blood transfusions and 9,779 transfusion complications from 194,432 blood donors were included in our dataset. We divided donors into three groups based on the number of complications and complication frequency (potentially “dangerous” vs. two differently defined control groups i.e. control 1 and control 2), and compared the nature of transfusion complications and demographic variables by statistical analysis. Results: There were no differences in the proportion of complication types between the potentially “dangerous” donors and control donors, and no difference in the proportion of complications from RBCs, plasma or platelets according to ABO and RhD blood types. However, more potentially “dangerous” donors were female and had ABO blood type B compared to control donors (p<0.001 and p<0.01, respectively). The potentially “dangerous” donors were younger compared to control donors (40.36 years vs. 45.24 years and 42.84 years, p<0.001). Conclusion: The potentially “dangerous” did not display more/severe immunologic transfusion complications compared to control donors. However, they differed in regards to gender, age and blood type. Further research regarding the differences in complication frequency per donor and demographic variety is warranted.

Fibrinolysis after an Elective Caesarean Section without Postpartum Hemorrhage: Pilot Study for Reference Values from the Simultaneous Thrombin and Plasmin Generation Assays

ntroduction: Postpartum Hemorrhage (PPH)-induced coagulopathy should be better explored. An innovative Simultaneous Generation of Thrombin and Plasmin Assay (SGTPA) was developed. Objective: To establish postpartum SGTPA references. Methods: Blood samples collected immediately after delivery (T0) and then 30, 60, 120 and 360 minutes later. Thrombin Generation (TG) and Plasmin Generation (PG) changes over time analyzed in 51 women after elective cesarean section without PPH and compared with Non-Pregnant (NP) women. The SGTPA variables’ correlations with fibrinogen levels, D-dimer levels and physiological blood loss were assessed in a mixed model. Results: 37 women were included. TG and PG were higher in the postpartum group than in the NP group. TG increased early and then remained stable (baseline TG Area Under the Curve (AUC) [95% Confidence Interval (CI)] = 41037 [36850-43537] nM.min). The fibrinogen level varied over time, along with TG (p<0.001). Plasmin generation increased from 30 to 120 minutes (AUC [95% CI]: 2104 [1437; 2613] nM.min), along with a change in the D-dimer level (p=0.018). The time to the plasmin peak and the time interval between the TG and PG peaks showed the greatest inter-individual variability at T0 and were associated with physiological volumes of blood loss during cesarean delivery. Conclusion: Reference SGTPA postpartum range was established. The SGTPA appears to be valuable for measuring TG and PG. TG and coagulation activation increased immediately after delivery, whereas PG and fibrinolysis increased later.

Bacterial Screening of Blood Components: Past, Present and Possibly Future Methodologies for Improving Transfusion Safetys

Major developments have been made in the past two decades to ensure that products are free from viral pathogens. Such improvements were achieved, mainly by the introduction of advanced screening methodologies such as Nucleic Acid Testing. Even though this has vastly improved transfusion safety, bacterial contamination remains a persistent threat. To mitigate this, improved donor arm disinfection, blood diversion pouches and proactive screening of blood components have been implemented by transfusion services all over the globe with great success. This review will focus on how current bacterial screening methodologies have improved over the years and how newer developments within this sector may further reduce the undesired possibility of transfusing contaminated blood products.

Metachronous Marginal Zone Lymphoma Followed by a Peripheral T-Cell Lymphoma in the Same Patient. Report of Two Cases and Review of the Literatures

Marginal Zone Lymphomas (MZL) and Peripheral T Cell Lymphomas (PTCL) are uncommon neoplasms and patients affected by both lymphomas in a lifetime have been rarely reported. Here we present two patients with an initial diagnosis of a MZL who further developed a PTCL. We also present the review of the literature of six other cases of MZL. Half of the cases reported are synchronous and the other half metachronous. Most MZLs reported are MALT subtype. There is no predominant subtype amongst PTCLs. Chronic exposure to proinflammatory cytokines, genetic predisposition or exposure to carcinogenic agents could be possible causes underlying this rare phenomenon.

The Effects of Chronic Inflammatory Disorders and Storage Lesions on Cytokine Levels in Blood Transfusion Productss

Whole blood donated for transfusion is processed into four blood products, namely Red Cell Concentrates (RCCs), Fresh Frozen Plasma, Buffy Coats (BCs), and Platelet Concentrates which are obtained by further processing of BCs. These blood products are then given to vulnerable patient cohorts such as anaemic, cancer or post-operative patients requiring RCCs. However, these blood products may contain biomolecules such as inflammatory mediators that may cause adverse yet often overlooked effects in the recipient. Chronic inflammation is characteristic of numerous diseases such as cardiovascular diseases, cancer, type 2 diabetes, rheumatoid arthritis, inflammatory bowel disease, asthma and psoriasis. Such diseases elevate pro-inflammatory cytokines including Interleukin (IL)-1, IL-6 and Tumour Necrosis Factor (TNF)- alpha in the blood. Moreover, pro-inflammatory cytokines also accumulate in blood products during storage, mainly as a result of damaged leucocytes, and may cause Transfusion-Associated Adverse Reactions (TAARs) and transfusion-induced systemic inflammation. Although this is well-known, very little scientific research has investigated the clinical implications and actual adverse effects these abnormal cytokine levels in blood products from donors with underlying chronic inflammatory diseases have on vulnerable recipients, particularly critically-ill patients who are already prone to inflammation.