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JPMA. the Journal of the Pakistan... Sep 2022To determine the aetiologies of pancytopenia based on bone trephine biopsy among paediatric and adult patients.
OBJECTIVE
To determine the aetiologies of pancytopenia based on bone trephine biopsy among paediatric and adult patients.
METHOD
The retrospective cross-sectional study was conducted at the Haematology Department of Aga Khan University Hospital, Karachi, and comprised data from June 1, 2016, to October 31, 2019 related to pancytopenia patients who underwent bone marrow biopsy. Data included age, gender, presenting symptoms, physical examination, complete blood count, peripheral smear, bone marrow aspirate and trephine biopsy findings and final diagnosis. Data was analysed using SPSS 19.
RESULTS
Of the 2852bone marrow biopsies done, 255(9%) related to evaluation of pancytopenia. Of them, 208(82%) were adult and 47(18%) were paediatric patients. The median age for adults was 38.8 years (range: 16-92years) and that in paediatric patients was 10.9 years (range: 2-15 years). Presenting symptoms were available for 182(71.4%) patients, and the commonest symptom was generalised weakness 128(70.3%). Overall, pallor was the most frequent sign 233(93.2%). Anisocytosis was predominant blood smear finding 156(61.1%), while the commonest aetiology was aplastic anaemia in both paediatric 23(49%) and adult 57(27.4%) groups. Bone marrow biopsy established the diagnosis in 253(99.2%) cases, while 2(0.95%) adult cases were not diagnosed. Of the diagnosed cases, 103(40.4%) were malignant; 15(32%) paediatric patients and 88(42.3%) adults. The rest were benign; 31(67.4%) paediatric patients and 119(3%) adults.
CONCLUSIONS
Bone marrow biopsy helped in diagnosing all but 2 pancytopenic patients. Aplastic anaemia was the commonest cause in both paediatric and adult patients.
Topics: Adult; Child; Humans; Adolescent; Young Adult; Middle Aged; Aged; Aged, 80 and over; Pancytopenia; Bone Marrow; Bone Marrow Examination; Anemia, Aplastic; Retrospective Studies; Cross-Sectional Studies; Biopsy
PubMed: 36280982
DOI: 10.47391/JPMA.2092 -
Haematologica Jan 2021
Topics: Bone Marrow Failure Disorders; Genomics; Humans; Pancytopenia
PubMed: 33386715
DOI: 10.3324/haematol.2020.265124 -
BMJ Case Reports Jul 2020A 21-year-old man presented to the emergency department with generalised weakness, weight loss and decreased appetite for few weeks. He had evidence of severe...
A 21-year-old man presented to the emergency department with generalised weakness, weight loss and decreased appetite for few weeks. He had evidence of severe pancytopenia and haemolysis. His peripheral smear with many schistocytes was suspicious for thrombotic thrombocytopenic purpura (TTP). He was supported with blood transfusions and daily plasmapheresis. His platelet counts worsened despite 4 days of therapy. Bone marrow biopsy was significant for hypercellular bone marrow with megaloblastic changes. Further workup revealed normal ADAMTS13 level, low vitamin B, positive intrinsic factor antibodies and high methylmalonic acid. Diagnosis of pernicious anaemia was established and he was started on daily treatment with intramuscular vitamin B which subsequently improved his symptoms and haematological parameters. This report highlights the importance of checking vitamin B level in patients presenting with pancytopenia and TTP-like picture before making a diagnosis of TTP.
Topics: Anemia, Pernicious; Humans; Male; Pancytopenia; Purpura, Thrombotic Thrombocytopenic; Young Adult
PubMed: 32624489
DOI: 10.1136/bcr-2020-235288 -
Environmental Health Perspectives Jul 1989The hematotoxicity of benzene exposure has been well known for a century. Benzene causes leukocytopenia, thrombocytopenia, pancytopenia, etc. The clinical and... (Review)
Review
The hematotoxicity of benzene exposure has been well known for a century. Benzene causes leukocytopenia, thrombocytopenia, pancytopenia, etc. The clinical and hematologic picture of aplastic anemia resulting from benzene exposure is not different from classical aplastic anemia; in some cases, mild bilirubinemia, changes in osmotic fragility, increase in lactic dehydrogenase and fecal urobilinogen, and occasionally some neurological abnormalities are found. Electromicroscopic findings in some cases of aplastic anemia with benzene exposure were similar to those observed by light microscopy. Benzene hepatitis-aplastic anemia syndrome was observed in a technician with benzene exposure. Ten months after occurrence of hepatitis B, a severe aplastic anemia developed. The first epidemiologic study proving the leukemogenicity of benzene was performed between 1967 and 1973 to 1974 among shoe workers in Istanbul. The incidence of leukemia was 13.59 per 100,000, which is a significant increase over that of leukemia in the general population. Following the prohibition and discontinuation of the use of benzene in Istanbul, there was a striking decrease in the number of leukemic shoe workers in Istanbul. In 23.7% of our series, consisting of 59 leukemic patients with benzene exposure, there was a preceding pancytopenic period. Furthermore, a familial connection was found in 10.2% of them. The 89.8% of our series showed the findings of acute leukemia. The possible factors that may determine the types of leukemia in benzene toxicity are discussed. The possible role of benzene exposure is presented in the development of malignant lymphoma, multiple myeloma, and lung cancer.
Topics: Anemia, Aplastic; Benzene; Hematologic Diseases; Humans; Leukemia; Neoplasms; Pancytopenia
PubMed: 2676498
DOI: 10.1289/ehp.8982193 -
Frontiers in Immunology 2022The characteristic feature of immune-related pancytopenia (IRP) is autoantibody-mediated bone marrow (BM) damage and peripheral blood cytopenia. We found that the...
The characteristic feature of immune-related pancytopenia (IRP) is autoantibody-mediated bone marrow (BM) damage and peripheral blood cytopenia. We found that the potential antigen of IRP was Ferritin light chain (FTL) by SEREX (serological analysis of recombinant cDNA expression libraries) in the previous study. In this study, we tried to explore the antigenic epitopes of FTL and verify its antigenicity in IRP. We found the possible FTL epitope: VNLYLQASYTYLSLG by phage random peptide library. Through ELISPOT, it was found that peptide VNLYLQASYTYLSLG can significantly stimulate the production of interleukin-4 and cannot stimulate the production of interferon-γ, which suggested that the peptide can obviously activate Th2 cells. Peptide-major histocompatibility complex tetramer elicited antigen-specific T cell responses. The expression levels of FTL were significantly increased in the patients with untreated IRP (P < 0.05). In conclusion, we found that FTL is the target antigen for some patients with IRP. The peptide of VNLYLQASYTYLSLG is an epitope of the target antigen. The target antigen is abnormally overexpressed on the membrane of BM cells, especially on the surface of CD34+ BM cells of patients with IRP. In addition, it is related to the severity of disease. These results provide a possible new target for the treatment of IRP in the future.
Topics: Apoferritins; Autoantigens; Bone Marrow; Epitopes; Humans; Pancytopenia
PubMed: 35572557
DOI: 10.3389/fimmu.2022.851096 -
The American Journal of Emergency... Sep 2021As the Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV2) pandemic progresses, various hematologic complications have emerged, often centered around the...
As the Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV2) pandemic progresses, various hematologic complications have emerged, often centered around the hypercoagulable state. However, pancytopenia represents a rare but serious complication from SARS-CoV2 infection. While lymphopenia is a common finding, concomitant acute anemia and thrombocytopenia are not commonly reported. We describe a novel case of SARS-CoV2 pancytopenia in a 40-year-old male without active risk factors for cell line derangements but subsequent critical illness.
Topics: Acute Kidney Injury; Adult; COVID-19; Continuous Renal Replacement Therapy; Humans; Male; Pancytopenia; Respiration, Artificial; Respiratory Insufficiency; SARS-CoV-2
PubMed: 33653644
DOI: 10.1016/j.ajem.2021.02.043 -
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 -
BMJ Case Reports Sep 2020A 35-year-old woman presented with a widespread petechial rash and pancytopenia. She underwent simultaneous pancreas and kidney transplantation for type 1 diabetes 8...
A 35-year-old woman presented with a widespread petechial rash and pancytopenia. She underwent simultaneous pancreas and kidney transplantation for type 1 diabetes 8 years previously followed by a renal transplant 1 year prior to presentation, and was taking tacrolimus as long-term immunosuppression. The full blood count showed haemoglobin 97 g/L, platelet count 2×10/L and neutrophil count 0.22×10/L. Peripheral blood film examination confirmed genuine thrombocytopenia in the absence of any haemolytic or malignant features. Serological testing identified autoantibodies against all three blood lineages, consistent with a diagnosis of autoimmune pancytopenia. Treatment with steroids, intravenous immunoglobulins, romiplostim and mycophenolate mofetil achieved only fleeting remissions. Blood counts eventually normalised following the administration of rituximab and a change from tacrolimus to ciclosporin immunosuppression. Cytopenias are a well-recognised complication of post-transplantation care but we believe this to be the first reported case of autoimmune pancytopenia following solid organ transplantation. In this case report, we discuss the approach to investigation of haematological abnormalities post-transplant and the rationale for, and outcome of, the management of this rare case.
Topics: Adult; Autoimmune Diseases; Female; Humans; Kidney Transplantation; Pancreas Transplantation; Pancytopenia; Postoperative Complications; Time Factors
PubMed: 32900732
DOI: 10.1136/bcr-2020-235851 -
The Primary Care Companion For CNS... Dec 2021
Topics: Humans; Intellectual Disability; Pancytopenia; Phenytoin; Sclerosis; Temporal Lobe; Valproic Acid; Young Adult
PubMed: 34942064
DOI: 10.4088/PCC.20cr02897 -
The Pan African Medical Journal 2019The aim of this study was to evaluate the severity of hematological findings according to etiology in patients with pancytopenia and bicytopenia.
INTRODUCTION
The aim of this study was to evaluate the severity of hematological findings according to etiology in patients with pancytopenia and bicytopenia.
METHODS
Patients with bicytopenia and pancytopenia who were examined in Diyarbakir Children Hospital Pediatric Hematology and Oncology clinic between June 2017-June 2018 were evaluated retrospectively.
RESULTS
Of the 130 patients included in the study, 73 (56.2%) were male and 43 (43.8%) were female. The mean age of the patients was 4.9 ± 4.86. Forty-five (34.6%) patients had pancytopenia and 85 (65.3%) had bicytopenia. The youngest patient was 1-month old and the oldest patient was 18-year-old. The mean blood count parameters were white blood cell (WBC): 10.207 ± 39.781, neutrophil: 1515 ± 1418, hgb: 9.3 ± 2.3, mean corpuscular volume (MCV): 80 ± 13.1, platelet: 118.823 ± 93.645. Three out of 130 patients had hyperleukocytosis (WBC > 50.000/mm). Vitamin B12 deficiency was detected in 35 patients. When patients with primary hematological disease were compared with patients with secondary causes of cytopenias, a significant difference was found in terms of leukocyte count, hemoglobin level, MCV elevation, and low platelet count.
CONCLUSION
The determination of the severity of cytopenias in differential diagnosis may be useful in distinguishing primary hematological diseases from secondary causes of pancytopenia and bicytopenia. However, vitamin B12 deficiency in developing countries is one of the most important causes of public health as well as in the etiology of pancytopenia and bicytopenia.
Topics: Adolescent; Child; Child, Preschool; Diagnosis, Differential; Erythrocyte Indices; Female; Hematologic Diseases; Humans; Infant; Leukocyte Count; Male; Neutrophils; Pancytopenia; Platelet Count; Retrospective Studies; Severity of Illness Index; Vitamin B 12 Deficiency
PubMed: 32110266
DOI: 10.11604/pamj.2019.34.149.18749