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The Lancet. Infectious Diseases Oct 2022
Topics: Exanthema; Fever; Humans; Pancytopenia
PubMed: 36152667
DOI: 10.1016/S1473-3099(22)00453-4 -
Journal of Family Medicine and Primary... Feb 2021Pancytopenia is a triage of anemia, leukopenia, and thrombocytopenia. The etiology causing pancytopenia varies depending upon factors such as age, sex, occupation, and...
BACKGROUND
Pancytopenia is a triage of anemia, leukopenia, and thrombocytopenia. The etiology causing pancytopenia varies depending upon factors such as age, sex, occupation, and geographical distribution. Unfortunately, the major treatises of hematology have not given emphasis on the hemorrhagic manifestation of different etiologies causing pancytopenia.
OBJECTIVE
This observational study was carried out with the aim to identify hemorrhagic manifestation in patients with pancytopenia in eastern India.
DESIGN
This study was conducted over a period of two years at the Department of Medicine of a tertiary care teaching institute in eastern India. All the patients with features of anemia, thrombocytopenia, or leukopenia were screened for pancytopenia and a total of 214 cases were selected. Patients were divided into two groups as patients with age more than 14 years constitute group one and the patients less than 14 years constitute the second group. A detailed physical examination, hematological, and biochemical investigation was done to ascertain the hemorrhagic manifestations in pancytopenia patients.
RESULTS
In the groups, the most common cause of hemorrhagic manifestation in patients with pancytopenia was aplastic anemic, leukemia, myelodysplastic syndrome, and myelofibrosis. No bleeding manifestation was seen in patients with megaloblastic anemia, kala-azar, hypersplenism, and other causes of pancytopenia.
CONCLUSIONS
Patients with pancytopenia caused by aplastic anemia, acute leukemia, and myelodysplastic syndrome have more chances of bleeding manifestation as compared with pancytopenia caused by megaloblastic anemia, kala-azar, or hypersplenism.
PubMed: 34041080
DOI: 10.4103/jfmpc.jfmpc_1117_20 -
Indian Journal of Dermatology,... 2023
Topics: Humans; Pancytopenia; Paraproteinemias
PubMed: 35146977
DOI: 10.25259/IJDVL_425_2021 -
Haematologica Oct 2023Clinical trials have shown that lentiviral-mediated gene therapy can ameliorate bone marrow failure (BMF) in nonconditioned Fanconi anemia (FA) patients resulting from...
Clinical trials have shown that lentiviral-mediated gene therapy can ameliorate bone marrow failure (BMF) in nonconditioned Fanconi anemia (FA) patients resulting from the proliferative advantage of corrected FA hematopoietic stem and progenitor cells (HSPC). However, it is not yet known if gene therapy can revert affected molecular pathways in diseased HSPC. Single-cell RNA sequencing was performed in chimeric populations of corrected and uncorrected HSPC co-existing in the BM of gene therapy-treated FA patients. Our study demonstrates that gene therapy reverts the transcriptional signature of FA HSPC, which then resemble the transcriptional program of healthy donor HSPC. This includes a down-regulated expression of TGF-β and p21, typically up-regulated in FA HSPC, and upregulation of DNA damage response and telomere maintenance pathways. Our results show for the first time the potential of gene therapy to rescue defects in the HSPC transcriptional program from patients with inherited diseases; in this case, in FA characterized by BMF and cancer predisposition.
Topics: Humans; Fanconi Anemia; Hematopoietic Stem Cells; Genetic Therapy; Transforming Growth Factor beta; Up-Regulation; Pancytopenia; Bone Marrow Failure Disorders
PubMed: 37021532
DOI: 10.3324/haematol.2022.282418 -
Expert Review of Hematology Nov 2022Copper is increasingly being recognized as a vital mineral required by both animals and humans. It plays a vital role in many metabolic processes such as cellular... (Review)
Review
INTRODUCTION
Copper is increasingly being recognized as a vital mineral required by both animals and humans. It plays a vital role in many metabolic processes such as cellular respiration, iron oxidation, and hemoglobin synthesis. Copper deficiency, which can be hereditary or acquired, can lead to a wide spectrum of disease processes such as ringed sideroblastic anemia, myelodysplasia, and pancytopenia. Timely identification and management of copper deficiency is necessary to prevent irreversible complications.
AREAS COVERED
Our study focuses on prevalence, etiology, pathophysiology, complications, and treatment of copper deficiency.
EXPERT OPINION
Copper deficiency is frequently underrecognized as the cause of anemia, neutropenia, and bone marrow dysplasia. As it is potentially treatable, it should always be kept in the differentials when patients present with neurological and hematological abnormalities.
Topics: Animals; Humans; Pancytopenia; Copper; Anemia; Hematologic Diseases; Neutropenia; Myelodysplastic Syndromes
PubMed: 36314081
DOI: 10.1080/17474086.2022.2142113 -
The National Medical Journal of India 2023Prolonged fever with pancytopenia and hepatosplenomegaly is a clinical entity frequently encountered by physicians. The diagnosis of such cases is challenging due to the...
Prolonged fever with pancytopenia and hepatosplenomegaly is a clinical entity frequently encountered by physicians. The diagnosis of such cases is challenging due to the diversity of differential diagnoses. Hepatosplenic T-cell lymphoma is a rare and aggressive type of non-Hodgkin lymphoma that can present with massive hepatosplenomegaly, pancytopenia and prolonged fever. Most of the patients are young men and the majority are associated with chronic immunosuppression. We report a 40-year-old immunocompetent woman with prolonged fever and pancytopenia due to hepatosplenic T-cell lymphoma.
Topics: Humans; Pancytopenia; Adult; Female; Splenomegaly; Hepatomegaly; Fever; Lymphoma, T-Cell; Splenic Neoplasms; Liver Neoplasms; Diagnosis, Differential
PubMed: 38692598
DOI: 10.25259/NMJI_207_20 -
Biomedicine & Pharmacotherapy =... Dec 2023Acquired aplastic anemia (AA) is a bone marrow failure (BMF) disease, characterized by fatty bone marrow (BM) and BM hypocellularity resulted from auto-immune...
Acquired aplastic anemia (AA) is a bone marrow failure (BMF) disease, characterized by fatty bone marrow (BM) and BM hypocellularity resulted from auto-immune dysregulated T cells-mediated destruction of BM haemopoietic stem cells (HPSC). The objective of this study was to investigate potential therapeutic effect of irisin, a molecule involved in adipose tissue transition, on AA mouse model. Our results showed that the concentration of irisin in serum was lower in AA patients than in healthy controls, suggesting a role of irisin in the pathogenesis of AA. In the AA mice, irisin administration prolonged the survival rate, prevented or attenuated peripheral pancytopenia, and preserved HPSC in the BM. Moreover, irisin also markedly reduced BM adipogenesis. In vitro results showed that irisin increased both cell proliferation and colony numbers of HPSC. Furthermore, our results demonstrated that irisin upregulated the expression of mitochondrial ATPase Inhibitory Factor 1 (IF1) in HPSC, inhibited the activation of mitochondrial fission protein (DRP1) and enhanced aerobic glycolysis. Taken together, our findings indicate novel roles of irisin in the pathogenesis of AA, and in the protection of HPSC through stimulation of proliferation and regulation of mitochondria function, which provides a proof-of-concept for the application of irisin in AA therapy.
Topics: Animals; Humans; Mice; Anemia, Aplastic; Bone Marrow; Bone Marrow Cells; Fibronectins; Pancytopenia; Hematopoietic Stem Cells
PubMed: 37952356
DOI: 10.1016/j.biopha.2023.115863 -
Blood Reviews Nov 2023Despite recent advancements, treatment of cytopenia due to bone marrow failures (BMF) and myeloid neoplasms remains challenging. Androgens promote renewal and maturation... (Review)
Review
Despite recent advancements, treatment of cytopenia due to bone marrow failures (BMF) and myeloid neoplasms remains challenging. Androgens promote renewal and maturation of blood cells and may be beneficial in these forms. Here we report a systematic review of androgens use as single agent in hematologic conditions. Forty-six studies, mainly retrospective with various androgen types and doses, were included: 12 on acquired aplastic anemia (AA), 11 on inherited BMF, 17 on myelodysplastic syndromes (MDS), and 7 on myelofibrosis. Responses ranged from 50 to 70% in inherited BMF, 40-50% in acquired AA and MDS, while very limited evidence emerged for myelofibrosis. In acquired AA, response was associated with presence of non-severe disease; in MDS androgens were more effective on thrombocytopenia or mild to moderate anemia, whilst limited benefit was observed for transfusion dependent anemia. Toxicity profile mainly consisted of virilization and liver enzyme elevation, whilst the risk of leukemic evolution remains controversial.
Topics: Humans; Androgens; Primary Myelofibrosis; Retrospective Studies; Neoplasms; Anemia, Aplastic; Myelodysplastic Syndromes; Bone Marrow Failure Disorders; Pancytopenia; Myeloproliferative Disorders; Thrombocytopenia
PubMed: 37709654
DOI: 10.1016/j.blre.2023.101132 -
Journal of Hematopathology Dec 2023A teenage girl presented with fevers of unknown origin and pancytopenia. Complete blood count showed anemia (hemoglobin, 9.0 g/dL), neutropenia (1.7 × 10/L), and...
A teenage girl presented with fevers of unknown origin and pancytopenia. Complete blood count showed anemia (hemoglobin, 9.0 g/dL), neutropenia (1.7 × 10/L), and thrombocytopenia (66 × 10/L). The bone marrow was hypocellular with left shifted hematopoiesis and myeloid hypoplasia. Aspirate smears were notable for a prominent population of neutrophils with crescentic nuclei that engulfed blue amorphous material (Fig. 1 panels A and B, Wright-Giemsa, magnification × 1000). The trephine biopsy showed similar cells with crescentic nuclei and eosinophilic material (Fig. 1 panels C and D, hematoxylin and eosin × 400). Flow cytometry was negative for an abnormal population. EBV by in situ hybridization and parvovirus immunohistochemistry were negative. Subsequent serologic testing was positive for ANA (1:1280), low C3/C4, anti-dsDNA, anti-SM and anti-B2GP1. A kidney biopsy demonstrated findings consistent with class III lupus nephritis.
Topics: Female; Adolescent; Humans; Neutrophils; Bone Marrow; Pancytopenia; Biopsy; Neutropenia
PubMed: 38175431
DOI: 10.1007/s12308-023-00561-8 -
Scandinavian Journal of Immunology Aug 2020Some patients with pancytopenia do not conform to any diagnostic criteria of known haematological or non-haematological diseases; however, they respond well to... (Review)
Review
Some patients with pancytopenia do not conform to any diagnostic criteria of known haematological or non-haematological diseases; however, they respond well to corticosteroid, high-dose intravenous immunoglobulin and rituximab treatment. This abnormality is termed immunorelated pancytopenia (IRP). Later studies indicated that IRP might be a kind of autoimmune disease in which T helper (Th) type 2 cell function is enhanced, resulting in the hyperfunction of B lymphocytes, which then produce excess autoantibodies that attack the bone marrow (BM) and cause cytopenia. Hypofunction of regulatory T (Treg) cells and enhanced Th17 cell function, an elevated percentage of plasmacytoid dendritic cells (pDCs) and a decreased percentage of natural killer (NK) cells help to promote the process. Moreover, increased expression of a synergistic stimulator of B lymphocytes, CD70 and the reactive overexpression of the BCR inhibitory coreceptor CD22 also support this claim. Candidate autoantigens targeted by autoantibodies on haematopoietic cell membranes have also been reported in IRP. This review is focused on studies that demonstrate the role of immune responses in the pathogenesis of IRP. Current diagnostic criteria and treatments for IRP are also referenced to provide a thorough understanding. Distinguishing IRP from idiopathic cytopenias of undetermined significance (ICUS) and other haematological disorders, for example myelodysplastic syndrome (MDS), aplastic anaemia (AA), paroxysmal nocturnal hemoglobinuria (PNH) and Evans syndrome, may help patients with pancytopenia benefit from proper treatment. Further studies are required to achieve new insight into the pathophysiology of IRP with regard to the immune system, which will be instrumental for the development of novel therapies for inhibiting disease initiation and/or progression.
Topics: Humans; Pancytopenia
PubMed: 32474938
DOI: 10.1111/sji.12911