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J Pediatr 2021 Apr 6. Epub 2021 Apr 6.

IRCCS Istituto Giannina Gaslini, Genoa, Italy; Università degli Studi di Genova, Genoa, Italy; Sechenov First Moscow State Medical University, Moscow, Russian Federation.

Objective: To describe the clinical characteristics, treatment, and outcomes of a multinational cohort of patients with macrophage activation syndrome (MAS) and thrombotic microangiopathy (TMA).

Study Design: International pediatric rheumatologists were asked to collect retrospectively the data of patients with the co-occurrence of MAS and TMA. Clinical and laboratory features of patients with systemic juvenile idiopathic arthritis (sJIA)-associated MAS and TMA were compared with those of an historical cohort of patients with sJIA and MAS. Read More

Pak J Pharm Sci 2020 Sep;33(5(Supplementary)):2269-2273

Imran Adrees College of Pharmacy, 3Km Daska Road, Sialkot, Pakistan.

Myelosuppression or bone marrow suppression is one of the most common side effects caused by anti-cancer drugs. Certain nonsteroidal anti-inflammatory drugs (NSAIDs), antibiotics and viruses like B19 virus can also cause bone marrow suppression resulting in serious consequences like leukopenia, anemia and thrombocytopenia. Currently, it is mainly treated by Filgrastim, use of which is not without side effects. Read More

J Infect 2021 Apr 5. Epub 2021 Apr 5.

ICMR-National Institute of Malaria Research, Sector 8, Dwarka, New-Delhi 110077, India. Electronic address:

Objectives: A systematic review and meta-analysis (SR-MA) of the available Indian literature on severe vivax malaria (SVM) was undertaken.

Methods: Relevant studies in eight electronic databases were retrieved and reviewed. The preferred reporting items for systematic reviews and meta-analyses (PRISMA) guidelines were followed. Read More

Rinsho Ketsueki 2021 ;62(3):176-179

Department of Hematology and Oncology, Mie University Hospital.

Acquired thrombotic thrombocytopenic purpura (aTTP) is a life-threatening systemic thrombotic microangiopathy characterized by the presence of anti-ADAMTS13 antibodies (inhibitor). Here we report the case of a patient with refractory aTTP successfully treated with cyclosporine. A 69-year-old man presenting with hematuria and petechiae was referred to our hospital; he was disoriented and febrile. Read More

BMC Infect Dis 2021 Apr 7;21(1):329. Epub 2021 Apr 7.

Department of Biomedical Science and Technology, School of Public Health and Community Development, Maseno University, Private Bag, Maseno, Kenya.

Background: In Plasmodium falciparum infection, clinical conditions such as anaemia, thrombocytopenia and leukocytosis are common. Mutation in haemoglobin sub-unit beta gene (HBB) may be a genetic factor responsible for these haematological changes during infection. However, the contributions of the carriage of different HBB genotypes on these changes remain largely unknown. Read More

BMC Pediatr 2021 Apr 6;21(1):161. Epub 2021 Apr 6.

Department of Neonatology, Center for Maternal-Fetal, Neonatal and Reproductive Medicine, National Center for Child Health and Development, 2-10-1 Okura, Setagaya-ku, Tokyo, 157-8535, Japan.

Background: Systemic juvenile xanthogranuloma is a very rare disease typically presents as skin lesions with yellow papules or nodules and is sometimes fatal. We report a case of congenital neonatal systemic juvenile xanthogranuloma with atypical skin appearance that made the diagnosis difficult.

Case Presentation: A preterm Japanese female neonate with prenatally diagnosed fetal hydrops in-utero was born with purpuric lesions involving the trunk and face. Read More

Folia Med Cracov 2020 ;60(4):53-64

Department of Hematology, Transplantation and Internal Medicine, Medical University of Warsaw, Poland.

Malignancies of the hematopoietic system frequently are associated with severe cytopenias requiring transfusions of blood components. Refusal of blood components by Jehovah's Witnesses (JW) produces challenges to treatment. In this report we describe the outcome of hematological malignancies of JW patients treated without transfusions. Read More

Expert Rev Endocrinol Metab 2021 Apr 5:1-7. Epub 2021 Apr 5.

Institute of Sport Sciences, Otto-von-Guericke University, Magdeburg, Germany.

: Changes in hematological parameters are becoming evident as important early markers of COVID-19. Type 2 Diabetes Mellitus (T2DM) has been shown to be associated with increased severity of COVID-19. In this study, we aim to explore the various hematological variables in COVID-19 positive patients with T2DM, so as to act early and improve patient outcomes. Read More

Indian J Sex Transm Dis AIDS 2020 Jul-Dec;41(2):156-161. Epub 2020 Jul 31.

Department of Community Medicine, PSG Institute of Medical Sciences and Research, Coimbatore, Tamil Nadu, India.

Background: A variety of hematological manifestations are seen at every stage of human immunodeficiency virus (HIV) infection, and they often pose a great challenge in the comprehensive management of acquired immunodeficiency syndrome. Anemia is the most common hematological abnormality associated with HIV infection. The severity and the incidence of cytopenia are usually correlated with the stage of the disease and underlying immune status if interpreted cautiously, especially if the patient is on regular follow-up. Read More

Zhongguo Shi Yan Xue Ye Xue Za Zhi 2021 Apr;29(2):456-461

Department of Hematology, The First Affiliated Hospital of Gannan Medical University, Ganzhou 341000，Jiangxi Province, China,E-mail:

Objective: To evaluate the clinical efficacy and safety of domestic imatinib (made in China) in patients with newly diagnosed chronic myeloid leukemia chronic phase(CML-CP).

Methods: Fifty-seven newly diagnosed CML-CP patients who did not receive any other anti-CML treatment were treated by domestic imatinib 400 mg once a day. The hematological, cytogenetic and molecular reactions and safety were observed and evaluated after 3, 6 and 12 months of treatment. Read More

Clin J Gastroenterol 2021 Apr 3. Epub 2021 Apr 3.

Department of Gastroenterology, Saitama Medical University International Medical Center, 1397-1, Yamane, Hidaka, Saitama, 350-1298, Japan.

A 76-year-old man diagnosed with early-stage colorectal cancer was referred to our hospital for endoscopic submucosal dissection (ESD). The patient had a low platelet count (31,000/µL) due to immune thrombocytopenia (ITP). The cancerous lesion was completely resected without any adverse events. Read More

Cancers (Basel) 2021 Mar 26;13(7). Epub 2021 Mar 26.

Hematology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, 20122 Milan, Italy.

Autoimmune cytopenias (AICy) and autoimmune diseases (AID) can complicate both lymphoid and myeloid neoplasms, and often represent a diagnostic and therapeutic challenge. While autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) are well known, other rarer AICy (autoimmune neutropenia, aplastic anemia, and pure red cell aplasia) and AID (systemic lupus erythematosus, rheumatoid arthritis, vasculitis, thyroiditis, and others) are poorly recognized. This review analyses the available literature of the last 30 years regarding the occurrence of AICy/AID in different onco-hematologic conditions. Read More

J Clin Med 2021 Mar 25;10(7). Epub 2021 Mar 25.

Department of General Biochemistry, Faculty of Biology and Environmental Protection, University of Lodz, Pomorska 141/143, 90-236 Lodz, Poland.

Chronic oxidative stress (OS) can be an important factor of acute myeloid leukemia (AML) progression; however, there are no data on the extent/consequence of OS after transfusion of packed red blood cells (pRBCs) and platelet concentrates (PCs), which are commonly used in the treatment of leukemia-associated anemia and thrombocytopenia. We aimed to investigate the effects of pRBC/PC transfusion on the OS markers, i.e. Read More

J Clin Med 2021 Mar 17;10(6). Epub 2021 Mar 17.

Pediatric Rheumatology, Institute of Rheumatology, Tokyo Women's Medical University, Tokyo 162-8666, Japan.

Antiphospholipid syndrome (APS) is a systemic autoimmune disorder mainly characterised by increased risks of thrombosis and pregnancy morbidity and persistent positive test results for antiphospholipid antibodies (aPLs). The criteria for diagnosing juvenile APS have yet to be validated, while the Sydney classification criteria do not contain several non-thrombotic clinical manifestations associated with the presence of aPLs. As such, difficulties have been encountered in the diagnosis of patients who have no certain thrombotic occlusions. Read More

J Clin Med 2021 Mar 2;10(5). Epub 2021 Mar 2.

Department of Hematology and Central Hematology Laboratory, Inselspital Bern, University of Bern, 3010 Bern, Switzerland.

Thrombocytopenia can arise from various conditions, including myelodysplastic syndromes (MDS) and bone marrow failure (BMF) syndromes. Meticulous assessment of the peripheral blood smear, identification of accompanying clinical conditions, and characterization of the clinical course are important for initial assessment of unexplained thrombocytopenia. Increased awareness is required to identify patients with suspected MDS or BMF, who are in need of further investigations by a step-wise approach. Read More

Front Oncol 2020 26;10:619625. Epub 2021 Feb 26.

Department of Oncology, The Second Affiliated Hospital of Nanchang University, Nanchang, China.

Purpose: To analyze the outcomes and toxicities of induction chemotherapy (ICT) followed by concurrent chemoradiotherapy (CCRT) plus adjuvant chemotherapy (ACT) in patients with locoregionally advanced nasopharyngeal carcinoma (LA-NPC).

Methods: Retrospective analysis of 163 patients with LA-NPC referred from August 2015 to December 2018 was carried out. All patients underwent platinum-based ICT followed by CCRT plus ACT. Read More

J Trop Pediatr 2021 01;67(1)

Department of Pediatrics and Neonatology, Max Super Speciality Hospital, Mohali, Punjab, India.

Lay Summary: Clinical and laboratory parameters of multisystem inflammatory syndrome in children (MIS-C) mimic Kawasaki disease (KD). KD has been described in association with dengue, scrub typhus and leptospirosis. However, MIS-C with concomitant infection has rarely been reported in literature. Read More

Medicine (Baltimore) 2021 Apr;100(13):e20866

Department of Pediatrics, Asan Medical Center Children's Hospital, University of Ulsan College of Medicine, Seoul.

Introduction: DOCK8 deficiency is a primary immunodeficiency characterized by recurrent infections, severe allergic disease, and autoimmunity. Here, we report a patient with DOCK8 deficiency that was initially presented as systemic lupus erythematosus (SLE) without recurrent infections and treated with hematopoietic stem cell transplantation (HSCT).

Patient Concerns: A 16-month-old boy with a previous history of eczema developed high fever and hand and foot swelling. Read More

Cancer Med 2021 Mar 30. Epub 2021 Mar 30.

Division of Hematology, Department of Internal Medicine, College of Medicine, National Cheng Kung University Hospital, National Cheng Kung University, Tainan, Taiwan.

Most acute myeloid leukemia (AML) cells are argininosuccinate synthetase-deficient. Pegylated arginine deiminase (ADI-PEG20) monotherapy depletes circulating arginine, thereby selectively inducing tumor cell death. ADI-PEG20 was shown to induce complete responses in ~10% of relapsed/refractory or poor-risk AML patients. Read More

Immunol Med 2021 Mar 30:1-4. Epub 2021 Mar 30.

Center for Postgraduate Education and Training, NCCHD, Tokyo, Japan.

Atypical hemolytic uremic syndrome (aHUS) is a rare disease caused by overactivation of the complement alternative pathway. aHUS involves the presence of antibodies against complement factor H and its mutations in the complement genes. A 2-month-old boy presented with discoid rash, hemolytic anemia, thrombocytopenia, multiple antibodies, and hypocomplementemia with a very low level of C4 (< 3 mg/dL), indicating activation of the complement pathway, together fulfilling the systemic lupus erythematosus (SLE) criteria of the American College of Rheumatology at 5 months of age. Read More

Cochrane Database Syst Rev 2021 Mar 23;3:CD012862. Epub 2021 Mar 23.

Renal Department, Newcastle University/Freeman Hospital, Newcastle Upon Tyne, UK.

Background: Atypical haemolytic uraemic syndrome (aHUS) is a rare disorder characterised by thrombocytopenia, microangiopathic haemolytic anaemia, and acute kidney injury. The condition is primarily caused by inherited or acquired dysregulation of complement regulatory proteins with ~40% of those affected aged < 18 years. Historically, kidney failure and death were common outcomes, however, improved understanding of the condition has led to discovery of novel therapies. Read More

Clin Nucl Med 2021 Mar 16. Epub 2021 Mar 16.

From the Division of Hematology/Oncology, Department of Pediatrics, School of Medicine Hematology Research Center, Amir Oncology Hospital, Shiraz University of Medical Sciences, Shiraz The Persian Gulf Nuclear Medicine Research Center, Department of Molecular Imaging and Radionuclide Therapy, Bushehr Medical University Hospital, Bushehr University of Medical Sciences, Bushehr Cancer Research Center, Razavi Hospital, Imam Reza International University, Mashhad Hematology Research Center, Shiraz University of Medical Sciences, Shiraz Department of Pediatric Hematology and Oncology, School of Medicine, Shahid Sadoughi University of Medical Sciences, Yazd, Iran Department of Diagnostic Imaging, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada Department of Nuclear Medicine, Klinikum Westfalen, Dortmund, Germany.

Background: Recent evidence has demonstrated high expression of somatostatin receptors in neuroblastoma (NB) cells. Because of this, we endeavored to evaluate the diagnostic performance and clinical efficacy of 68Ga-DOTATATE PET/CT and peptide receptor radionuclide therapy (PRRT) using 177Lu-DOTATATE combined with chemotherapy in pediatric NB patients.

Patients And Methods: In total, 14 pediatric patients with histopathologically confirmed NB underwent 68Ga-DOTATATE PET/CT. Read More

Transplant Cell Ther 2021 Mar 16;27(3):260.e1-260.e6. Epub 2020 Dec 16.

Department of Oncology, Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins University School of Medicine, Baltimore, Maryland. Electronic address:

Large granular lymphocytosis (LGL)-or LGL leukemia-is a T- or NK-cell lymphoproliferative disorder that often results in cytopenias and autoimmune phenomena. Several studies have described LGL in a subset of patients after allogeneic blood or marrow transplantation (alloBMT), almost exclusively in the setting of asymptomatic lymphocytosis. Some have suggested an association with improved transplant-related outcomes. Read More

Arthritis Rheumatol 2021 Mar 28. Epub 2021 Mar 28.

National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, USA.

Objective: Somatic mutations in ubiquitin activating enzyme 1 (UBA1) cause a newly defined syndrome known as VEXAS. More than fifty percent of patients currently identified with VEXAS meet diagnostic criteria for relapsing polychondritis (RP). Clinical features that characterize VEXAS within a cohort of RP have not been defined. Read More

Urol Oncol 2021 Mar 25. Epub 2021 Mar 25.

Department of Urology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Kita-Ku, Okayama, Japan.

Purpose: This study aimed to clarify the efficacy and toxicity of first-line combination treatment with paclitaxel, cisplatin, and gemcitabine (PCG) for advanced/metastatic urothelial carcinoma (UC) in cisplatin-unfit patients compared with cisplatin-fit patients.

Methods: We conducted a retrospective study of patients who received first-line PCG. Using international consensus criteria, patients were classified into cisplatin-fit and -unfit groups. Read More

J Thromb Haemost 2021 Mar 28. Epub 2021 Mar 28.

Department of Clinical Laboratory, University of Yamanashi Hospital, Chuo, Yamanashi, Japan.

Background: Erythropoiesis is a complex multistep process by which erythrocytes are produced. C-type lectin-like receptor 2 (CLEC-2) is a podoplanin (PDPN) receptor almost exclusively expressed on the surface of platelets and megakaryocytes. Deletion of megakaryocyte/platelet CLEC-2 was reported to cause anemia along with thrombocytopenia in mice. Read More

Int J Hematol 2021 Mar 27. Epub 2021 Mar 27.

Nagoya City West Medical Center Hematology and Oncology, Nagoya, Japan.

Malignant lymphoma developing during anti-PD-1 antibody treatment is extremely rare. A 74-year-old female was admitted with left hypochondrial pain. She was diagnosed with squamous cell carcinoma of the right upper lobe of the lung, and had undergone surgery and postoperative chemotherapy three years prior. Read More

Int J Hematol 2021 Mar 27. Epub 2021 Mar 27.

Pediatric Onco-Hematology Department, La Paz University Hospital, Madrid, Spain.

Severe aplastic anemia and congenital amegakaryocytic thrombocytopenia are rare bone marrow failure syndromes. Treatment for aplastic anemia consists of hematopoietic stem cell transplantation (HSCT) from a matched sibling donor or immunosuppressant drugs if there is no donor available. Congenital amegakaryocytic thrombocytopenia is a rare autosomal recessive disease that causes bone marrow failure and has limited treatment options, except for transfusion support and HSCT. Read More

J Pediatr Hematol Oncol 2021 Mar 23. Epub 2021 Mar 23.

Department of Pathology and Laboratory Medicine Department of Pediatrics, Division of Hematology, Oncology, and Stem Cell Transplantation, Ann and Robert H. Lurie Children's Hospital, Chicago, IL Department of Pathology, Indiana University School of Medicine, Indianapolis, IN.

The diagnostic criteria for juvenile myelomonocytic leukemia have recently been revised to include clinical findings and RAS-pathway gene mutations per the 2016 World Health Organization Classification of Tumors of Hematopoietic and Lymphoid Tissues. Differing clinical behaviors have been observed in cases with CBL versus other RAS-pathway gene (RAS-p) mutations, notably the patients with CBL mutations can be self-limiting with spontaneous resolution. Additional clinical characteristics and histopathologic findings between these subsets are less well-described. Read More

Clin Nephrol 2021 Mar 26. Epub 2021 Mar 26.

A 60-year-old Japanese woman with polymyositis (PM) developed hemolytic anemia (hemoglobin of 7.3 g/dL), thrombocytopenia (platelet of 9.1×10/µL), and acute kidney injury (Cre of 4. Read More