Hematology Reports

10 pages, 588 KiB

Open AccessArticle

Bisphosphonate Use for Glucocorticoid-Induced Osteoporosis in Elderly Patients with Immune Thrombocytopenia Receiving Prolonged Steroid Therapy: A Single Institute Retrospective Study

by Satoshi Yamasaki, Kenjiro Kamezaki, Yoshikiyo Ito and Takahiko Horiuchi

Hematol. Rep. 2022, 14(3), 276-285; https://doi.org/10.3390/hematolrep14030039 - 19 Sep 2022

Cited by 2 | Viewed by 2081

Abstract

Prednisolone, used as a standard initial treatment for immune thrombocytopenia (ITP), is an important risk factor for osteoporosis. To investigate the prevention of glucocorticoid-induced osteoporosis (GIO) in elderly ITP patients receiving prolonged steroid therapy, associations between GIO prevention and the real-world data of [...] Read more.

Prednisolone, used as a standard initial treatment for immune thrombocytopenia (ITP), is an important risk factor for osteoporosis. To investigate the prevention of glucocorticoid-induced osteoporosis (GIO) in elderly ITP patients receiving prolonged steroid therapy, associations between GIO prevention and the real-world data of score changes of a dual-energy X-ray absorptiometry (DXA) scan, FRAX^® and the Garvan tool during the initial loading of prednisolone were examined. In our institute, 22 ITP patients aged ≥ 70 years received 0.5–1.0 mg/kg prednisolone for 2–3 weeks as the initial ITP treatment between 2014 and 2021. The femoral neck bone mineral density (BMD) measured by DXA scan was entered into FRAX^® to define the risk-adapted approach to bisphosphonate during the initial loading of prednisolone. Bisphosphonate was administered according to <−1.0 femoral neck BMD T-score measured by DXA scan. Worse scores of FRAX^® and the Garvan tool were associated with bisphosphonate use for short-term fracture prevention in primary GIO; however, there were no incidents of fracture or significant differences in probabilities determined by FRAX^® and the Garvan tool. During the initial loading of prednisolone, prescribing bisphosphonate might prevent the reduction in BMD in elderly patients with ITP receiving prolonged steroid therapy. Full article

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6 pages, 2125 KiB

Open AccessCase Report

Congenital Neutropenia with Specific Granulocyte Deficiency Caused by Novel Double Heterozygous SMARCD2 Mutations

by Abukhiran Ibrahim, Anjali Sharathkumar, Heather McLaughlin, David Claassen and Sharathkumar Bhagavathi

Hematol. Rep. 2022, 14(3), 270-275; https://doi.org/10.3390/hematolrep14030038 - 09 Sep 2022

Cited by 1 | Viewed by 1646

Abstract

SMARCD2 (SWI/SNF-related, matrix-associated, actin-dependent regulator of chromatin, subfamily D, member 2) is critical for myelopoiesis. Recently, bi-allelic SMARCD2 mutations have been reported in five children, causing autosomal recessive congenital neutropenia with specific granulocytes deficiency (CN-SGD); a syndrome resulting in G-CSF resistant neutropenia, recurrent [...] Read more.

SMARCD2 (SWI/SNF-related, matrix-associated, actin-dependent regulator of chromatin, subfamily D, member 2) is critical for myelopoiesis. Recently, bi-allelic SMARCD2 mutations have been reported in five children, causing autosomal recessive congenital neutropenia with specific granulocytes deficiency (CN-SGD); a syndrome resulting in G-CSF resistant neutropenia, recurrent infections, and dysplastic myelopoiesis. We report a new case with CN-SGD caused by two novel heterozygous pathogenic variants in the SMARCD2 gene (c.1081del (p.Gln361Argfs*15)), and (c.217C>T (p.Arg73*)). Treatment with the weekly dosing of thrombopoietin receptor agonist, Romiplostim, along with daily G-CSF transformed her clinical course, implying potential synergism. This report advances the understanding of CN-SGD caused by SMARCD2 mutations. Full article

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5 pages, 433 KiB

Open AccessBrief Report

Triple-Negativity Identifies a Subgroup of Patients with Better Overall Survival in Essential Thrombocythemia

by Marco Santoro, Vincenzo Accurso, Salvatrice Mancuso, Mariasanta Napolitano, Marta Mattana, Giorgia Vajana, Federica Russello and Sergio Siragusa

Hematol. Rep. 2022, 14(3), 265-269; https://doi.org/10.3390/hematolrep14030037 - 24 Aug 2022

Cited by 1 | Viewed by 2395

Abstract

Essential thrombocythemia, as defined by the WHO in 2016, is a Philadelphia-negative chronic myeloproliferative neoplasm showing a better prognosis than polycythemia vera and myelofibrosis. In a variable percentage, patients with essential thrombocythemia show none of the known driver-gene mutations that may occur on [...] Read more.

Essential thrombocythemia, as defined by the WHO in 2016, is a Philadelphia-negative chronic myeloproliferative neoplasm showing a better prognosis than polycythemia vera and myelofibrosis. In a variable percentage, patients with essential thrombocythemia show none of the known driver-gene mutations that may occur on JAK2, CALR, and MPL genes. Such patients are classified as triple-negative and their clinical features and prognosis have not been described with precision yet. In this study, we evaluated some of the characteristics of this population by comparing them with those of patients with driver-gene mutated ET. Data from 266 consecutive essential thrombocythemia patients were analysed. Triple-negative patients had a significantly lower symptom load and a lower frequency of splenomegaly at diagnosis. The results show that the rate of thrombosis was equal in the two subgroups. Overall survival was slightly better in the triple-negative group of patients. Full article

(This article belongs to the Special Issue Personalized Therapy and Clinical Outcomes for Congenital and Acquired Haemorrhagic Disorders, Thromboembolic Disease and Platelet Disorders)

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4 pages, 425 KiB

Open AccessCase Report

Successful Treatment of Autoimmune Hemolytic Anemia Concomitant with Proliferation of Epstein-Barr Virus in a Post-Heart Transplant Patient

by Dan Ran Castillo, Parthiv Sheth, Kevin Nishino, Wesley Tait Stevens, Anthony Nguyen, Alberto Romagnolo and Hamid Mirshahidi

Hematol. Rep. 2022, 14(3), 261-264; https://doi.org/10.3390/hematolrep14030036 - 17 Aug 2022

Cited by 1 | Viewed by 2132

Abstract

Autoimmune hemolytic anemia (AIHA) is a rare complication following heart transplantation and has been attributed to several etiologies including infections, immunosuppressive medications, and post-transplant lymphoproliferative disorders. We report a 23-year-old male presenting 22 years after heart transplantation with severe AIHA. Laboratory findings were [...] Read more.

Autoimmune hemolytic anemia (AIHA) is a rare complication following heart transplantation and has been attributed to several etiologies including infections, immunosuppressive medications, and post-transplant lymphoproliferative disorders. We report a 23-year-old male presenting 22 years after heart transplantation with severe AIHA. Laboratory findings were notable for positive IgG autoantibody against RBCs and high titer Epstein-Barr virus (EBV) viremia. Shortly after the first unit of irradiated RBC transfusion and high dose steroids, the patient developed acute dyspnea and hypoxia requiring intubation. Further workup demonstrated that the patient had Methicillin-sensitive Staphylococcus aureus (MSSA) pneumonia (PNA) and bacteremia, requiring antibiotics. Patient was subsequently treated with high-dose steroids, IVIG, as well as rituximab. Following treatment, the patient was successfully extubated and eventually showed complete resolution of the anemia. This case is novel as it represents AIHA likely secondary to EBV viremia in a post-cardiac transplant patient complicated by a severe transfusion reaction. In this circumstance, rituximab in conjunction with standard of care remains an effective treatment of choice. Full article

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8 pages, 526 KiB

Open AccessReview

COVID-19-Associated Thrombotic Thrombocytopenic Purpura: A Case Report and Systematic Review

by Haseeb Chaudhary, Usama Nasir, Khezar Syed, Maria Labra, Christopher Reggio, Ansar Aziz, Parin Shah, Roopika Reddy and Navdeep Sangha

Hematol. Rep. 2022, 14(3), 253-260; https://doi.org/10.3390/hematolrep14030035 - 02 Aug 2022

Cited by 8 | Viewed by 2599

Abstract

Introduction: The proliferation of literature regarding the COVID-19 pandemic has served to highlight a wide spectrum of disease manifestations and complications, such as thrombotic microangiopathies. Our review with a brief case presentation highlights the increasing recognition of TTP in COVID-19 and describes its [...] Read more.

Introduction: The proliferation of literature regarding the COVID-19 pandemic has served to highlight a wide spectrum of disease manifestations and complications, such as thrombotic microangiopathies. Our review with a brief case presentation highlights the increasing recognition of TTP in COVID-19 and describes its salient characteristics. Methods: We screened the available literature in PubMed, EMBASE, and Cochrane databases from inception until April 2022 of articles mentioning COVID-19-associated TTP in English language. Results: From 404 records, we included 8 articles mentioning data of 11 patients in our review. TTP was predominantly reported in females (72%) with a mean age of 48.2 years (SD 15.1). Dyspnea was the most common symptom in one third of patients (36.6%). Neurological symptoms were reported in 27.3% of cases. The time to diagnosis of TTP was 10 days (SD 5.8) from onset of COVID-19. All 11 cases underwent plasma exchange (PLEX), with a mean of 12 sessions per patient, whereas 6 cases received Rituximab (54.5%), and 3 received Caplacizumab (27.3%). One patient died from the illness. Conclusion: This review of available literature highlights the atypical and refractory nature of COVID-19-associated TTP. It required longer sessions of PLEX, with half of the patients receiving at least one immunosuppressant. Full article

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8 pages, 419 KiB

Open AccessArticle

Clinical Epidemiology Characteristics and Etiology of Febrile Neutropenia in Children: Analysis of 421 Cases

by Sang Ngoc Nguyen, Lam Tung Vu, Quang Van Vu, Tham Thi Tran and Vi Tuong Thi Dinh

Hematol. Rep. 2022, 14(3), 245-252; https://doi.org/10.3390/hematolrep14030034 - 01 Aug 2022

Cited by 3 | Viewed by 2745

Abstract

The congenital immune system includes neutrophils, which perform a variety of functions. Congenital and acquired neutropenia are rare illnesses with an underestimated prevalence in children. The aim of this study is to examine the epidemiology and etiology of febrile neutropenia in children at [...] Read more.

The congenital immune system includes neutrophils, which perform a variety of functions. Congenital and acquired neutropenia are rare illnesses with an underestimated prevalence in children. The aim of this study is to examine the epidemiology and etiology of febrile neutropenia in children at Haiphong Children’s Hospital, Haiphong, Vietnam. Methods: A cross-sectional study was carried out on 421 febrile neutropenia children. Clinical and laboratory characteristics were examined. Results: The median age (IQR) was 25.0 (12.5–59.5) months. The male-to-female ratio was 1.35/1. There were twice as many children living in the suburbs (66.98%) as in urban areas (33.02%). The mean (SD) temperature at admission was 38.50 ± 0.59 °C. Diagnosed causes associated with neutropenia included acute respiratory infections 250 (59.45%), gastrointestinal infections 68 (16.1%), erythema 37 (8.79%), acute leukemia 15 (3.56%), urinary tract infection 5 (1.19%), and encephalitis/meningitis 4 (0.95%). Viral etiology accounted for 61.52% (259): influenza type A—50.19% (130), influenza type B—31.27% (81), dengue virus—14.67% (38), measles virus 1—93% (5), rotavirus—1.54% (4), and EBV—0.4% (1). Twenty-five patients (5.94%) were found to have bacteria in their cultures, with Streptococcus pneumonia being the most common (eight patients; 32%). Conclusions: Febrile neutropenia was common in children under 2 years old. Primary clinical manifestations were acute upper respiratory tract infections, and viruses most commonly caused febrile neutropenia. Further studies with larger sample sizes are needed to determine the cause of febrile neutropenia. Full article

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5 pages, 1209 KiB

Open AccessCase Report

Efficacy of Eltrombopag in Children with Post-Stem Cell Transplant Prolonged Isolated Thrombocytopenia

by Megumi Matsumoto, Kazuki Terada, Taichiro Tsuchimochi, Satoko Takahashi, Yasushi Noguchi and Shunji Igarashi

Hematol. Rep. 2022, 14(3), 240-244; https://doi.org/10.3390/hematolrep14030033 - 01 Aug 2022

Cited by 1 | Viewed by 1745

Abstract

Prolonged isolated thrombocytopenia (PIT) is a complication following allogeneic hematopoietic cell transplantation that results in prolonged transfusion dependence. Recently, the efficacy of a thrombopoietin receptor agonist (eltrombopag) against PIT has been reported in adults; however, there are few reports in children. A 4-year-old [...] Read more.

Prolonged isolated thrombocytopenia (PIT) is a complication following allogeneic hematopoietic cell transplantation that results in prolonged transfusion dependence. Recently, the efficacy of a thrombopoietin receptor agonist (eltrombopag) against PIT has been reported in adults; however, there are few reports in children. A 4-year-old male pediatric patient diagnosed with congenital pure red cell aplasia underwent allogeneic hematopoietic cell transplantation. Neutrophil engraftment was observed on post-transplant Day 26; however, platelet counts remained <10 × 10⁹/L. Transfusions were required 1–2 times a week for at least 4 months. On post-transplant Day 124, oral eltrombopag (up to 2.4 mg/kg/day) was initiated. Thereafter, the platelet counts were maintained at ≥10 × 10⁹/L, and the patient became transfusion independent. At 2 years and 6 months after the oral administration, no chromosomal abnormalities, thromboembolism, or myelofibrosis was observed. Thus, eltrombopag can be a potential treatment option for pediatric PIT. Full article

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5 pages, 228 KiB

Open AccessCase Report

Acute Hyperhemolysis Syndrome in a Patient with Known Sickle Cell Anemia Refractory to Steroids and IVIG Treated with Tocilizumab and Erythropoietin: A Case Report and Review of Literature

by Sasmith R. Menakuru, Adelina Priscu, Vijaypal Dhillon and Ahmed Salih

Hematol. Rep. 2022, 14(3), 235-239; https://doi.org/10.3390/hematolrep14030032 - 21 Jul 2022

Cited by 12 | Viewed by 2739

Abstract

Patients with sickle cell anemia often receive multiple red blood cell (RBC) transfusions during their lifetime. Hyperhemolysis is a life-threatening phenomenon of accelerated hemolysis and worsening anemia that occurs when both transfused RBCs and autologous RBCs are destroyed. The level of hemoglobin post-transfusion [...] Read more.

Patients with sickle cell anemia often receive multiple red blood cell (RBC) transfusions during their lifetime. Hyperhemolysis is a life-threatening phenomenon of accelerated hemolysis and worsening anemia that occurs when both transfused RBCs and autologous RBCs are destroyed. The level of hemoglobin post-transfusion is lower than pre-transfusion levels, and patients are usually hemodynamically unstable. Hyperhemolysis must be differentiated from a delayed hemolytic transfusion reaction during which destruction of transfused RBC is the cause of anemia. Hyperhemolysis syndrome can be differentiated into acute (within seven days) and chronic forms (after seven days) post-transfusion. The authors present a case of acute hyperhemolysis syndrome in a patient with sickle cell anemia refractory to steroids and IVIG, which are the treatment of choice. The patient was treated with tocilizumab, combined with supportive measures of erythropoietin, iron, vitamin B12, and folate. Full article

7 pages, 1031 KiB

Open AccessArticle

Hematologic Complications in Patients Hospitalized with COVID-19 Infection

by Elisa Lin, Ellen Araj, John Markantonis, Hung Luu and Mingyi Chen

Hematol. Rep. 2022, 14(3), 228-234; https://doi.org/10.3390/hematolrep14030031 - 11 Jul 2022

Cited by 1 | Viewed by 3110

Abstract

Introduction: This review summarizes data from patients with COVID-19 requiring intensive care unit (ICU) admission. The goals of this study are to showcase some morphological anomalies found in peripheral blood smears from COVID-19 patients and to bring attention to how some hematologic abnormalities [...] Read more.

Introduction: This review summarizes data from patients with COVID-19 requiring intensive care unit (ICU) admission. The goals of this study are to showcase some morphological anomalies found in peripheral blood smears from COVID-19 patients and to bring attention to how some hematologic abnormalities in COVID-19 that correspond to disease severity and mortality. Methods: We performed a retrospective analysis of hematologic parameters using peripheral blood smear analysis from 31 COVID-19 patients hospitalized between April 2021 and January 2022. Results: We found abnormal morphology that has not been previously reported. We also report that severe lymphopenia, neutrophilia, acute hemolysis, hematologic malignancies, and increased LDH are associated with ICU admissions, respiratory failure requiring intubation, and poor clinical outcome. Conclusion: We propose these recommendations in the management of COVID-19 patients: 1. Early diagnosis and follow-up of DIC; 2. Optimization of thromboprophylaxis regimen. Full article

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6 pages, 234 KiB

Open AccessArticle

Comparison of Response to Rituximab Therapy in Adults with Refractory Symptomatic Immune Thrombocytopenia According to the Presence of Accessory Spleen

by Fehmi Hindilerden, Ipek Yonal-Hindilerden, Mustafa Nuri Yenerel, Meliha Nalcaci and Reyhan Diz-Kucukkaya

Hematol. Rep. 2022, 14(3), 222-227; https://doi.org/10.3390/hematolrep14030030 - 05 Jul 2022

Viewed by 1863

Abstract

No data exist for the association between the presence of accessory spleen after splenectomy and response to rituximab in immune thrombocytopenia (ITP). We investigated the relationship between accessory spleen presence and rituximab response in splenectomized ITP patients. Fifteen chronic refractory ITP patients were [...] Read more.

No data exist for the association between the presence of accessory spleen after splenectomy and response to rituximab in immune thrombocytopenia (ITP). We investigated the relationship between accessory spleen presence and rituximab response in splenectomized ITP patients. Fifteen chronic refractory ITP patients were included. Four weekly doses of rituximab 375 mg/m² were administered. All patients had undergone splenectomy before rituximab administration. Accessory spleen was detected in 5 of 15 patients (33.3%). Median age at diagnosis was significantly higher in patients with accessory spleen than those without accessory spleen (40 (range 25–68 years) and 26 (range 7–40 years), respectively; p = 0.049). There was a trend for older age at time of rituximab initiation in patients with accessory spleen compared to the other group (median 51 (range 43–75 years) and 42.5 (range 30–60 years), respectively; p = 0.066). Median follow-up duration was 96 months (range 40–98). We demonstrated a significant correlation between accessory spleen presence and older age. Accessory spleen presence correlated with higher platelet and WBC counts. We showed good inverse correlation between presence of accessory spleen and time to early response (ER) to rituximab while the rate of early response (ER), late response (LR), sustained response (SR) and overall response (OR) did not differ with respect to the presence of acessory spleen. Full article

(This article belongs to the Special Issue Personalized Therapy and Clinical Outcomes for Congenital and Acquired Haemorrhagic Disorders, Thromboembolic Disease and Platelet Disorders)

9 pages, 1388 KiB

Open AccessCase Report

CMV Colitis: A Rare Complication of Azacitidine and Venetoclax Chemotherapy

by Mustafa Nissar Bankur, Archie Keeling, Khoodoruth Mohamed Adil Shah and Daniele Avenoso

Hematol. Rep. 2022, 14(3), 213-221; https://doi.org/10.3390/hematolrep14030029 - 23 Jun 2022

Cited by 1 | Viewed by 2381

Abstract

Herein, we present a case of cytomegalovirus (CMV) colitis that occurred after two cycles of azacitidine and venetoclax in a 64-year-old woman affected with acute myeloid leukaemia (AML) secondary to a previous diagnosis of a hypoplastic myelodysplastic syndrome (hypo-MDS). This patient never had [...] Read more.

Herein, we present a case of cytomegalovirus (CMV) colitis that occurred after two cycles of azacitidine and venetoclax in a 64-year-old woman affected with acute myeloid leukaemia (AML) secondary to a previous diagnosis of a hypoplastic myelodysplastic syndrome (hypo-MDS). This patient never had detectable CMV viraemia, and there was no evidence of immune deficiency that could justify this opportunistic infection. Additionally, this is most likely the first report describing CMV colitis in a patient treated upfront with azacitidine and venetoclax. Full article

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3 pages, 198 KiB

Open AccessCase Report

Severe Hemolytic Anemia due to Vitamin B12 Deficiency in Six Months

by Narayanan Sadagopan

Hematol. Rep. 2022, 14(3), 210-212; https://doi.org/10.3390/hematolrep14030028 - 22 Jun 2022

Cited by 4 | Viewed by 2274

Abstract

Gastric bypass is a common cause of vitamin B12 deficiency. It can lead to patients presenting with symptoms of anemia. The body has significant reserves of vitamin B12 and loses vitamin B12 slowly. The following case is of a patient who underwent a [...] Read more.

Gastric bypass is a common cause of vitamin B12 deficiency. It can lead to patients presenting with symptoms of anemia. The body has significant reserves of vitamin B12 and loses vitamin B12 slowly. The following case is of a patient who underwent a gastric bypass five years ago and whose hemoglobin (Hgb) dropped from 12.2 g/dL to 4.4 g/dL over six months due to questionable adherence to vitamin supplements. Further work-up showed hemolytic anemia and thrombocytopenia due to a very low vitamin B12 level of 47 pg/mL, with his blood counts improving with vitamin B12 supplementation. The case points to the importance of thinking about vitamin deficiency as a cause of hemolysis to avoid unnecessary procedures. Full article

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Hematol. Rep., Volume 14, Issue 3 (September 2022) – 12 articles

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