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Hematology (Amsterdam, Netherlands) Dec 2019Pancytopenia is a frequent entity in clinical practice as a feature of a myriad of conditions, ranging from benign to malignant diseases. Since the cause of pancytopenia... (Review)
Review
BACKGROUND
Pancytopenia is a frequent entity in clinical practice as a feature of a myriad of conditions, ranging from benign to malignant diseases. Since the cause of pancytopenia depends on environmental factors, it is important to know the common etiologies of pancytopenia, however, few studies address this.
OBJECTIVES
To identify the etiology of pancytopenia in our population and compare them with what is reported elsewhere.
METHODS
We conducted an observational study of patients with pancytopenia in a Mexican Tertiary Care Center. Clinical, hematological and bone marrow studies were performed in all patients.
RESULTS
Of 109 cases included, the mean age at diagnosis was 49.4 years, with a slightly higher female incidence (53.2%). The most common causes of pancytopenia were: MDS (20.2%), megaloblastic anemia (18.3%) and AML (12.8%).
DISCUSSION
We found a complex picture of pancytopenia in Mexico and compared it with what is reported elsewhere in the literature.
CONCLUSION
The sociocultural context in which the patients develop helps narrowing the possible etiology of pancytopenia, and therefore hasten the diagnostic process. Of all the studies available, bone marrow aspiration seems the most useful.
Topics: Adolescent; Adult; Age of Onset; Aged; Aged, 80 and over; Female; Humans; Male; Mexico; Middle Aged; Pancytopenia; Sex Factors; Tertiary Care Centers
PubMed: 30890036
DOI: 10.1080/16078454.2019.1590961 -
Rheumatology International May 2024Aplastic anemia (AA) is a rare, potentially catastrophic hematopoiesis failure manifested by pancytopenia and bone marrow aplasia. AA occurrence in Systemic Lupus... (Review)
Review
Aplastic anemia (AA) is a rare, potentially catastrophic hematopoiesis failure manifested by pancytopenia and bone marrow aplasia. AA occurrence in Systemic Lupus Erythematosus (SLE) patients is extremely rare. The diagnosis may be delayed due to other possible pancytopenia etiologies. Confirmation of peripheral cytopenias diagnosis necessitates a bone marrow aspiration. The management of AA is challenging, and the literature reported using glucocorticoids, danazol, plasmapheresis, cyclophosphamide, intravenous immunoglobulin, and cyclosporine. We report two cases of SLE patients who presented with pancytopenia, with bone marrow biopsy confirmed AA. One case was treated with cyclophosphamide but unfortunately succumbed to Acute Respiratory Distress Syndrome (ARDS), while the other case was managed with rituximab with a good response. Interestingly, both patients were on azathioprine before the diagnosis of AA. A comprehensive search for reported cases of AA in PubMed, Scopus, and the Directory of Open Access Journals databases was performed to enhance the understanding of the diagnostic and management challenges associated with AA in SLE, facilitating ongoing exploration and research in this field. The decision to do a BM aspiration and biopsy is recommended for SLE patients with an abrupt decline in blood counts and previously stable blood counts.
Topics: Humans; Anemia, Aplastic; Pancytopenia; Lupus Erythematosus, Systemic; Cyclosporine; Cyclophosphamide
PubMed: 38512478
DOI: 10.1007/s00296-024-05585-6 -
European Journal of Haematology Aug 2023Aplastic anemia (AA) is a disease of bone marrow hematopoietic failure, and the main clinical manifestation is pancytopenia. Its pathogenesis is still unclear. In recent... (Review)
Review
Aplastic anemia (AA) is a disease of bone marrow hematopoietic failure, and the main clinical manifestation is pancytopenia. Its pathogenesis is still unclear. In recent years, more research has been done on its immune abnormalities to explain its pathogenesis and less on the hematopoietic microenvironment, but there are still some advances. This article summarizes the research on the hematopoietic microenvironment of AA in recent years to provide new ideas for the clinical treatment of AA.
Topics: Humans; Anemia, Aplastic; Hematopoietic Stem Cells; Pancytopenia
PubMed: 37203325
DOI: 10.1111/ejh.13991 -
Journal of Gastroenterology and... Nov 2022
Review
Topics: Female; Humans; Middle Aged; Hypergammaglobulinemia; Pancytopenia; Plasma Cells; Immunocompromised Host; Gastrointestinal Tract
PubMed: 35288972
DOI: 10.1111/jgh.15820 -
BMJ Case Reports May 2022A female child hailing from South Asia, India presented with pallor, multiple petechiae and ecchymosis. Based on the clinical picture and demography, the differentials...
A female child hailing from South Asia, India presented with pallor, multiple petechiae and ecchymosis. Based on the clinical picture and demography, the differentials considered were pancytopenia of nutritional origin, acute leukaemia, autoimmune and infective aetiologies. After ruling these out by respective tests, a literature review was done which revealed the possibility of filariasis especially in a patient with eosinophilia which was present in our case. A repeat peripheral blood smear study with a nocturnally drawn sample revealed multiple microfilariae and a diagnosis of filariasis was made. The patient was treated with triple drug therapy of diethylcarbamazine (6 mg/kg), ivermectin (6 µg/kg) and albendazole (400 mg) administered as a single dose. Subsequent haemograms showed improved cell counts. This along with a previous handful of case reports emphasises filariasis as one of the differentials of pancytopenia and should be kept in mind while evaluating for the same, especially in the endemic areas.
Topics: Albendazole; Animals; Child; Diethylcarbamazine; Drug Therapy, Combination; Elephantiasis, Filarial; Female; Filaricides; Humans; Ivermectin; Pancytopenia; Wuchereria bancrofti
PubMed: 35606041
DOI: 10.1136/bcr-2022-248930 -
Pathology, Research and Practice Jul 2019Unexplained cytopenia is one of the most common indications for performing trephine bone marrow (BM) biopsy (BMB). The histopathological examination in this regard must... (Review)
Review
Unexplained cytopenia is one of the most common indications for performing trephine bone marrow (BM) biopsy (BMB). The histopathological examination in this regard must be seen in the broader context of a multimodal approach in order to reach an as entity-specific as possible diagnosis, considering medical history, physical examination, laboratory data, peripheral blood morphology, BM aspiration smear, flow cytometry results and, if indicated, cytogenetics and molecular genetics. The particular irreplaceability of the histopathological work-up and the expectations to the BMB lie especially in the detection of fibrosing and/or focal processes (e.g. localized islets of blasts) and disorders extrinsic to the BM such as e.g. metastases, thrombotic microangiopathies, granulomatous myelitides etc. We propose a systematic combined histopathological pattern-based and blood count-based approach that can be applied in such circumstances to achieve a precise diagnosis or, at least, a clinically useful differential diagnosis, particularly taking into consideration specific morphologic pitfalls and application of ancillary techniques. Constitutional BM failure syndromes will not be profoundly addressed.
Topics: Biopsy; Bone Marrow; Flow Cytometry; Humans; Pancytopenia
PubMed: 31101575
DOI: 10.1016/j.prp.2019.152447 -
Folia Medica Mar 2019Low-dose once weekly methotrexate (MTX) is the first-line disease-modifying antirheumatic drug (DMARD) to treat rheumatoid arthritis and psoriasis. Although methotrexate...
Low-dose once weekly methotrexate (MTX) is the first-line disease-modifying antirheumatic drug (DMARD) to treat rheumatoid arthritis and psoriasis. Although methotrexate is generally considered to have a good safety profile it can occasionally induce severe side effects such as pancytopenia, mucositis, disorders of kidney and liver. Oral mucositis should alert physicians to MTX toxicity. We report a 64-year-old woman with a severe drug reaction including disseminated shingles, oral mucositis and pancytopenia only three days after starting a therapeutic low-dose of MTX. Initially, mucositis and myelosuppression couldn't be accounted for by the underlying disease. In taking the patient's history, MTX intake 10 days ago was missed, the patient reporting only current medications. However, there was more to the skin rash than meets the eye. Only after further inquiry did the patient reveal the intake of 2 doses of MTX and the subsequent withdrawal of medication. Arriving at the correct diagnosis in difficult cases, as in the case presented, requires further evaluation, including repeat history taking and eliciting more details if diagnosis remains elusive.
Topics: Arthritis, Rheumatoid; Female; Herpes Zoster; Humans; Methotrexate; Middle Aged; Pancytopenia; Stomatitis
PubMed: 31237855
DOI: 10.2478/folmed-2018-0046 -
The American Journal of the Medical... Feb 2022Methotrexate (MTX) is an effective medication in the treatment of rheumatoid arthritis (RA), other rheumatic diseases and various solid tumors. However, its side... (Review)
Review
Methotrexate (MTX) is an effective medication in the treatment of rheumatoid arthritis (RA), other rheumatic diseases and various solid tumors. However, its side effects, including gastrointestinal discomfort, oral ulcers, and especially bone marrow suppression, could be fatal and require special attention, particularly in patients with renal failure. We present two hemodialysis patients with RA who presented with a complication of severe pancytopenia after treatment with MTX. After receiving various supportive and blood purification treatments, both patients recovered. We reviewed twenty-four pancytopenia patients on dialysis associated with methotrexate. Among these patients, high morbidity and mortality were observed, indicating that MTX should be used cautiously in the absence of alternatives in such a population. Compared with the patients who recovered, the deceased patients showed a lower level of leukocytes. Which dialysis method might be the best choice is unclear. The mode of renal replacement therapy can be chosen according to the actual situation.
Topics: Antirheumatic Agents; Arthritis, Rheumatoid; Humans; Methotrexate; Pancytopenia; Renal Dialysis
PubMed: 34562416
DOI: 10.1016/j.amjms.2021.08.008 -
European Journal of Internal Medicine Oct 2022
Topics: Autoimmune Diseases; Chronic Disease; Epstein-Barr Virus Infections; Exanthema; Humans; Pancytopenia
PubMed: 35798590
DOI: 10.1016/j.ejim.2022.06.021 -
The Journal of Allergy and Clinical... Mar 2019Hemophagocytic lymphohistiocytosis (HLH) is an overwhelming clinical syndrome associated with extreme immune activation. Familial HLH is caused by autosomal-recessive... (Review)
Review
Hemophagocytic lymphohistiocytosis (HLH) is an overwhelming clinical syndrome associated with extreme immune activation. Familial HLH is caused by autosomal-recessive inheritance of gene mutations that cripple lymphocyte cytotoxicity. X-linked lymphoproliferative diseases and mutations in Nod-like receptor caspase activation and recruitment domain containing protein 4 (NLRC4) also feature HLH as a predominant manifestation. In addition, "secondary" HLH may occur in immunocompromized patients or in individuals with previously intact immune responses in the context of strong immunologic triggers such as EBV infection, malignancy, rheumatologic disease, and drug hypersensitivity. Regardless of the etiology, HLH is often fatal unless recognized and treated aggressively. Research over the last 20 years has led to many advances in diagnosis and treatment. Rapid testing strategies designed to quickly screen for immune activation and cytotoxic lymphocyte dysfunction are now clinically available and genetic panels/testing algorithms may accelerate a genetic diagnosis. Immunosuppressive treatment protocols have been refined, and experience is gaining with alternative and salvage approaches. However, these advances improve the outcome of patients only when the diagnosis of HLH is made. Ongoing education is needed to ensure medical providers can appropriately recognize and diagnose HLH. This Grand Rounds Review will summarize the clinical and diagnostic features of HLH and highlight known genetic causes.
Topics: Failure to Thrive; Female; Fever; Humans; Infant; Liver Diseases; Lymphohistiocytosis, Hemophagocytic; Mutation; Pancytopenia
PubMed: 30557712
DOI: 10.1016/j.jaip.2018.11.050