Platelet Transfusion: An Update on Indications and Guidelines

Platelets are commonly transfused either therapeutically or prophylactically to maintain hemostasis. Most platelet transfusions are used to manage patients with hematologic malignancies. Although platelet transfusion guidelines have been published, platelet transfusion practices are still heterogeneous. Platelet transfusion guidelines partly lack recommendations or differ in the platelet threshold recommendations in some clinical situations. This article reviews platelet transfusions focusing on transfusion guidelines and platelet thresholds in different clinical settings

Clinico-Laboratory Profile and Outcome of COVID-19 in Patients with Chronic Immune Thrombocytopenia

Background/Case Studies: Thrombocytopenia is a well-described complication of COVID-19, with numerous proposed mechanisms among which is immune thrombocytopenia (ITP). There are limited data on the characteristics and impact of COVID-19 on patients with previously diagnosed ITP. Study Design/Methods: This is a retrospective review of all chronic ITP patients who were diagnosed with COVID-19 between 03/2020 and 01/2022 at a tertiary care center. The study was approved by the IRB. Patients with secondary thrombocytopenia, missing data, and unavailable follow up after COVID-19 diagnosis, were excluded. Demographic data, comorbidities, clinico-laboratory findings before and after COVID-19 diagnosis, management of COVID-19 and outcome were collected. Analyses were performed using SPSS; a p value of \u3c0.05 was considered significant. Results were presented as median plus range, mean +/- standard deviation, or percentages as indicated. Variables were compared using the independent two-sample Student t-test for continuous variables, and the Pearson\u27s Chi-square test or Fisher\u27s exact test for categorical variables. Early mortality was defined as death from any cause within 30 days of admission. Results/Findings: 45 patients were included. The median age was 66 (32–93) years; 27 (60%) were females. 28(62%) patients were Caucasian. The median time from ITP diagnosis to COVID-19 was 5 (1–35) years. A 27 (60%) patients required treatment for ITP before COVID-19, and only 4 patients were on low-dose prednisone at the time of COVID-19 diagnosis. The most common symptoms of COVID-19 were shortness of breath (53%), fever (31%), and cough (22%). 29 (64%) patients were hospitalized with 12 of them requiring ICU care. Median time of hospitalization was 8 (2–45) days. COVID-19 specific treatments included steroids (42%), remdesivir (24%), chloroquine (9%), azithromycin (9%), and tocilizumab (2%). Three patients had thrombosis (2 DVTs, and 1 DVT and PE), 2 had intracranial bleeding, and 3 had mucosal bleeding. Early mortality rate was 15.6%; death was attributed to respiratory failure in 3 patients, multi-organ failure in 3 patients, and cardiac arrest in 1 patient. None of the analyzed parameters (gender, ethnicity, age, comorbidities, severity of thrombocytopenia, thrombotic or bleeding events) was associated with ICU admission or early mortality. Patients\u27 platelet count before COVID-19 diagnosis did not differ from the platelet count at the time of COVID-19 diagnosis with a mean platelet count of 108.5 (+/- 49.0) and 93.8 (+/- 92.8) (p = 0.299), respectively. In addition, there was no significant difference in the platelet before COVID-19 and after recovery. Conclusion: Thrombocytopenia of chronic ITP patients did not worsen during COVID-19 infection or after recovery. Mortality of chronic ITP patients due to COVID-19 was not different from reported mortality of hospitalized COVID-19 patients. Our findings should be validated in larger cohorts

Pharmacologic Therapies to Prevent Relapse of Acute Myeloid Leukemia After Allogeneic Hematopoietic Stem Cell Transplantation

Relapse is the main cause of mortality in patients with acute myeloid leukemia (AML) after allogeneic hematopoietic stem cell transplantation (allo-HSCT). Adverse cytogenetic or molecular risk factors, as well as refractory disease or persistent measurable residual disease (MRD) at the time of transplantation are associated with an increased risk of recurrence. Salvage therapy for AML relapse after allo-HSCT is often limited to chemotherapy, donor lymphocyte infusions and/or second transplants and is rarely successful. Effective post-transplant preventive intervention in high risk AML may be crucial. The most frequent and promising approach is the use of post-transplant maintenance with hypomethylating agents or with FLT3 tyrosine kinase inhibitors when the target is present. Moreover, IDH1/IDH2 inhibitors and BCL-2 inhibitors in combination with other strategies are promising approaches in the maintenance setting. Here we summarize the current knowledge about the preemptive and prophylactic use of pharmacologic agents after allo-HSCT to prevent relapse of AML

Non-Hodgkin's lymphoma in a woman with adult-onset Still's disease: a case report

INTRODUCTION: Adult onset Still's disease is a chronic multisystemic inflammatory disorder characterized by high spiking fever, polyarthralgia and rash. Lymphadenopathy is a prominent feature of adult onset Still's disease and is seen in about 65% of patients. Searching the medical literature using the MEDLINE database from January 1966 through November 2007 we could only find two reported cases of adult onset Still's disease that had progressed to lymphoma. CASE PRESENTATION: We describe a woman who was diagnosed with adult onset Still's disease and developed lymphoma 10 months after the onset of her symptoms. She initially presented with fever and arthritis of the knees, ankles and shoulders, along with a nonpruritic skin rash, myalgia and weight loss. On physical examination she was found to have several enlarged anterior cervical lymph nodes and left posterior auricular lymph nodes all of which were non-tender, immobile and rubbery. Excisional biopsy of the cervical lymph nodes was negative for malignancy. Bone marrow biopsy was also negative for malignancy. She was treated with prednisone. She remained in good health until she presented 10 months later with low back pain, dyspnea and weight loss. Work up revealed malignant lymphoma. She was treated with chemotherapy and was doing well until she presented with abdominal pain. Work up revealed a cirrhotic liver and ascites. She then passed away from hepatorenal syndrome 13 years after the diagnosis of lymphoma. To our knowledge, this is the third reported case of such an occurrence. CONCLUSION: Although the association between adult onset Still's disease and lymphoma has been rarely reported, careful screening for this malignancy in patients suspected to have adult onset Still's disease is warranted

Unilateral anterior uveitis complicating zoledronic acid therapy in breast cancer

BACKGROUND: Zoledronic acid is very widely used in patients with metastatic bone disease and osteoporosis. Only one case of bilateral uveitis was recently reported related to its use. CASE PRESENTATION: We report the first case of severe unilateral anterior uveitis in a patient with breast cancer and an intraocular lens. Following zoledronic acid infusion, the patient developed severe and dramatic right eye pain with decreased visual acuity within 24 hours and was found to have a fibrinous anterior uveitis of moderate severity The patient was treated with topical prednisone and atropine eyedrops and recovered slowly over several months. CONCLUSION: Internists, oncologists, endocrinologists, and ophtalmologists should be aware of uveitis as a possible complication of zoledronic acid therapy. Patients should be instructed to report immediately to their physicians and treatment with topical prednisone and atropine eyedrops should be instituted immediately at the onset of symptoms. This report documents anterior uveitis as a complication of zoledronic acid therapy. This reaction could be an idiosyncratic one but further research may shed more light on the etiology

Extracorporeal photopheresis: A case of graft-versus-host-disease and hemophagocytic lymphohistiocytosis following liver transplantation

BACKGROUND: Graft-versus-host-disease (GVHD) is one of the rare complications following liver transplantation. We report on the efficacy and safety of extracorporeal photopheresis (ECP) in managing GVHD and hemophagocytic lymphohistiocytosis (HLH) after liver transplantation. CASE REPORT: The patient is a 63-year-old male with hepatitis B cirrhosis who underwent liver transplantation. Three weeks after transplant, he presented with fever, diarrhea, and poor appetite. The patient also had bilateral blanchable erythematous patches on his palms, biopsy of which was suggestive of GVHD. The patient continued to have high-grade fever with altered mental status. CBC showed pancytopenia. Liver function examination was normal. Patient was started on methylprednisolone. Additional laboratory analysis showed high ferritin (\u3e15000 ug/L), triglycerides (280 mg/dl), and low fibrinogen (80 mg/dl). Chimerism analysis using short tandem repeat (STR) PCR confirmed the diagnosis of GVHD. Marrow biopsy showed hemophagocytosis. The patient fulfilled the HLH-2004 diagnostic criteria. He was kept on tacrolimus and steroids and was started on etanercept and ECP. After the first two cycles of ECP (one cycle defined as the weekly two procedures of ECP), the patient reported improvement of symptoms. He tolerated ECP well. His labs improved during the course of treatment, until his peripheral blood STR showed 100% recipient DNA. He was discharged after the fourth cycle of ECP to receive the remaining treatments as outpatient. At one year follow-up, the patient is asymptomatic with no evidence of GVHD or HLH. DISCUSSION: ECP in combination with immunosuppressive therapy and etanercept was safe and efficient in managing GVHD and HLH following liver transplantation

Platelet Transfusion: An Update on Indications and Guidelines

Platelets are commonly transfused either therapeutically or prophylactically to maintain hemostasis. Most platelet transfusions are used to manage patients with hematologic malignancies. Although platelet transfusion guidelines have been published, platelet transfusion practices are still heterogeneous. Platelet transfusion guidelines partly lack recommendations or differ in the platelet threshold recommendations in some clinical situations. This article reviews platelet transfusions focusing on transfusion guidelines and platelet thresholds in different clinical settings

Red cell exchange for acute splenic sequestration crisis in an adult with hemoglobin sc disease.

Purpose Acute splenic sequestration crisis (ASSC) is a well-known life-threatening complication of sickle cell disease in children. It is characterized by progressive splenic enlargement, severe anemia requiring multiple transfusions, and ultimately circulatory compromise. Modalities of management include red blood cell (RBC) transfusion, pain control, and splenectomy. ASSC has rarely been reported in adults, thus making its management and outcome in adults not clearly defined. The purpose of this report is to describe the favorable outcome of ASSC in an adult female with hemoglobin (Hb) SC disease managed with RBC exchange, thus avoiding a scheduled splenectomy. Methods Case report and literature review Results A 39-year-old African American lady with history of Hb SC disease, diagnosed at age of 10, presented to the surgical outpatient clinic with 1-month history of progressive abdominal swelling, abdominal pain and easy satiety. She was evaluated by her hematologist and was found to have markedly enlarged spleen on imaging measuring 20cm in its longest dimension raising the suspicion for ASSC. As a result, she has been given multiple blood transfusions over the past month with transient mild improvement of symptoms. Her past medical history was significant for a similar episode of abdominal pain at the age of 16, which responded to blood transfusion and pain management. Splenectomy was recommended in light of persistence of patient\u27s symptoms despite management with transfusions and pain medications. In preparation for splenectomy, the transfusion medicine service was consulted to perform one session of RBC exchange few days before surgery. Patient\u27s laboratory values on admission were Hb 8.7 g/dL, hematocrit 25.9%, WBC 3.8 × 109/L, MCV 81.2 fL, and platelets 105 × 109/L. Hb electrophoresis analysis revealed a combined Hgb S and C of 91%. RBC exchange was performed using the COBE Spectra apheresis system (COBE Spectra, Terumo BCT, Inc., Lakewood, CO) processing 2 blood volumes and replacing with fresh (\u3c10 days old), leukoreduced, sicklecell negative, C, E and K antigen-negative packed RBCs. We aimed for end hematocrit of 30% and end Hb S1C of 20%. We thought that the patient might respond to RBC exchange, so we recommended holding on splenectomy to evaluate the patient for response to RBC exchange. The patient tolerated RBC exchange very well; however her post procedure period was complicated by hypotensive episodes which were responsive to adequate hydration. Repeat evaluation after exchange revealed Hb 9.1g/dl and Hb electrophoresis showed Hb S1C of 29.8%. The patient started to feel better after RBC exchange and she could eat without discomfort. Her spleen was barely palpable below the left costal margin, and there was a marked reduction in splenic size on repeat imaging to 17cm four days after exchange. At 2-month follow up, the patient was free of symptoms and she did not require further blood transfusions. She was being considered for long term hydroxyurea 1/2 RBC exchange as outpatient. Conclusion Red cell exchange is used to replace sickle cells with normal RBCs. There are rare case reports on the role of exchange transfusion in adults with Hb SC disease. RBC exchange should be considered in adults with Hb SC disease with ASSC; a procedure that could improve patient outcome and avert complications from splenectomy especially in young patients