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Journal of Pediatric Hematology/oncology Mar 2021
Topics: Adolescent; Anemia; Copper; Female; Humans; Lymphopenia; Neutropenia; Pancytopenia; Prognosis
PubMed: 33448718
DOI: 10.1097/MPH.0000000000002047 -
Indian Journal of Gastroenterology :... Apr 2023Pancytopenia in children with celiac disease (CeD) is postulated to be due to nutritional deficiency such as vitamin B, folate and copper or an autoimmune process...
Pancytopenia in children with celiac disease (CeD) is postulated to be due to nutritional deficiency such as vitamin B, folate and copper or an autoimmune process resulting in aplastic anemia with hypoplastic marrow. In the present case series, we report the profile and explore the etiology of pancytopenia among children with CeD. There are only a few case reports of pancytopenia in children with CeD. We enrolled newly diagnosed cases of CeD and pancytopenia presenting in the celiac disease clinic over three years. Detailed evaluation was carried out for the cause of pancytopenia. We followed up on the cases for compliance and response to gluten-free diet at three months, six months and 12 months. Twenty patients were eligible for inclusion. They were divided into two groups: one with aplastic anemia with hypoplastic marrow labeled as Gp CeD-AA and the other with megaloblastic/nutritional anemia labeled as Gp CeD-MA. Patients in Gp CeD-MA presented with classical symptoms of CeD as recurrent diarrhea, abdomen distension, pallor and poor weight gain. They had none or just one transfusion requirement and had an early and complete recovery from pancytopenia. Patients in Gp CeD-AA presented with atypical symptoms such as epistaxis, short stature, fever, pallor and weakness. They had a multiple blood transfusion requirement and had delayed and partial recovery from pancytopenia. Pancytopenia is not a disease in itself but is the presentation of an underlying disease. It can occur due to various coexisting disorders in children with CeD, which can be as simple as nutritional deficiencies to as complex as an autoimmune process or malignancy. CeD should be included in the differential diagnosis of aplastic anemia as CeD and aplastic anemia both have a similar pathological process involving T cell destruction of tissues.
Topics: Humans; Child; Pancytopenia; Anemia, Aplastic; Celiac Disease; Pallor; Anemia, Megaloblastic
PubMed: 37162701
DOI: 10.1007/s12664-022-01327-3 -
International Journal of Molecular... Sep 2023We previously reported that granulocytic myeloid-derived suppressor cells (G-MDSCs) suppressed T-cell activation and attenuated bone marrow failure (BMF) in a minor...
We previously reported that granulocytic myeloid-derived suppressor cells (G-MDSCs) suppressed T-cell activation and attenuated bone marrow failure (BMF) in a minor histocompatibility (minor-H) antigen mismatched murine aplastic anemia (AA) model. In the current study, we tested the hypothesis that exosomes, a subset of extracellular vesicles, are responsible at least partially for G-MDSCs' therapeutic efficacy. Indeed, exosomes isolated from GMDSCs (G-MDSC-exos) suppressed CD4 and CD8 T-cell proliferation in vitro and mildly attenuated immune BMF in the minor-H mismatched AA model. G-MDSC-exos treatment significantly increased red blood cells, hemoglobin, and total bone marrow (BM) cells, and moderately reduced BM CD8 T cells. G-MDSC-exos' effects were associated with upregulations in an array of lymphocyte-suppression-related miRNAs such as hsa-miR-142-5p, miR-19a-3p, and miR-19b-3p in both BM CD4 and CD8 T cells. We concluded that G-MDSC-exos attenuate immune BMF via modulating the delivery of immunosuppressive miRNAs into activated T lymphocytes.
Topics: Mice; Animals; Myeloid-Derived Suppressor Cells; CD8-Positive T-Lymphocytes; Disease Models, Animal; Exosomes; Granulocytes; Immunosuppressive Agents; MicroRNAs; Pancytopenia; Bone Marrow Failure Disorders
PubMed: 37834110
DOI: 10.3390/ijms241914661 -
Journal of Veterinary Emergency and... Mar 2022(1) To report an unusual etiology for nontraumatic hemoabdomen in cats, and (2) to describe onset and recovery from severe, unexpected pancytopenia seen after surgical...
OBJECTIVE
(1) To report an unusual etiology for nontraumatic hemoabdomen in cats, and (2) to describe onset and recovery from severe, unexpected pancytopenia seen after surgical removal of a large intra-abdominal myelolipoma.
CASE SUMMARY
A 14-year-old neutered male domestic shorthair cat was presented for emergent treatment of suspected nontraumatic hemoabdomen. A hyperechoic mass, with ultrasonographic echogenicity similar to fat, was found in the right cranial abdomen and believed to be associated with the mesentery. Cytological examination of abdominal fluid identified marked extramedullary hematopoiesis within the hemorrhagic effusion. Exploratory laparotomy identified a hepatic mass, which was resected, and revealed to be a hepatic myelolipoma on histopathological examination. The patient's initial recovery was uneventful. However, continued hyporexia resulted in readmission 4 days postoperatively, at which time the patient was found to have a profound, tri-lineage pancytopenia, and cytological evidence indicative of bone marrow recovery. The pancytopenia resolved with continued medical management and supportive care.
NEW OR UNIQUE INFORMATION PROVIDED
Ruptured myelolipoma is not a commonly considered differential for nontraumatic hemoabdomen in cats. Furthermore, severe pancytopenia is unexpected following surgical resection of a myelolipoma. This case provides a unique clinical presentation of both nontraumatic hemoabdomen and bone marrow recovery.
Topics: Abdomen; Adrenal Gland Neoplasms; Animals; Cat Diseases; Cats; Hemoperitoneum; Male; Myelolipoma; Pancytopenia
PubMed: 34766713
DOI: 10.1111/vec.13157 -
Pediatrics in Review Oct 2023
Topics: Male; Humans; Adolescent; Pancytopenia
PubMed: 37777217
DOI: 10.1542/pir.2021-005494 -
The Pan African Medical Journal 2022Autoimmune lymphoproliferative syndrome (ALPS) is a rare genetic disorder of lymphocyte homeostasis, resulting from mutations in the Fas apoptotic pathway. It is...
Autoimmune lymphoproliferative syndrome (ALPS) is a rare genetic disorder of lymphocyte homeostasis, resulting from mutations in the Fas apoptotic pathway. It is characterized by non-infectious and non-malignant chronic lymphoproliferation and an increased risk of lymphoid malignancy. The diagnosis of this condition usually combines chronic lymphadenopathy and/or splenomegaly exceeding 6 months, autoimmune cytopenias, with an elevated level of CD3+CD4-CD8- Tαβ lymphocytes, known as "double-negative" T cells. Differential diagnosis includes infections, autoimmune diseases or malignancies. Although clinical examination and laboratory tests are highly suggestive, this disease goes widely unrecognized. We here report, for the first time, the case of ALPS, a Moroccan patient, and aged 8 years, with recurrent fever, splenomegaly and adenopathies. Paraclinical examinations revealed chronic pancytopenia, higher than normal TαΠdouble negative lymphocytes, hypergammaglobulinemia, and elevated serum levels of soluble FAS ligand. The diagnosis of ALPS was made. First-line treatment included corticosteroids and immunoglobulins. Then the patient received mycophenolate followed by Sirolimus. This treatment resulted in better clinical and laboratory tests results. Our aim is to raise awareness of this rare condition, which may be under-diagnosed, among physicians.
Topics: Humans; Autoimmune Lymphoproliferative Syndrome; Splenomegaly; Autoimmune Diseases; Sirolimus; Pancytopenia; Immunosuppressive Agents
PubMed: 36523284
DOI: 10.11604/pamj.2022.43.61.33009 -
Internal Medicine (Tokyo, Japan) Mar 2020We examined a 22-year-old woman who was admitted to our hospital with abdominal distention. At 19 years of age, the patient presented with hepatosplenomegaly. She was...
We examined a 22-year-old woman who was admitted to our hospital with abdominal distention. At 19 years of age, the patient presented with hepatosplenomegaly. She was examined several times in another hospital; however, the cause was unidentified. Our evaluation showed severe pancytopenia and a spleen 13×24 cm in size. The serum levels of angiotensin-converting enzyme and lysozyme were elevated. She was diagnosed with liver sarcoidosis based on non-caseating epithelioid granuloma in liver biopsy tissue. To improve the symptoms, splenectomy was performed, and her pancytopenia and symptoms improved. Sarcoidosis should be considered in cases of massive splenomegaly.
Topics: Biopsy; Female; Granuloma; Humans; Muramidase; Pancytopenia; Sarcoidosis; Spleen; Splenomegaly; Young Adult
PubMed: 31666466
DOI: 10.2169/internalmedicine.3646-19 -
Blood Cells, Molecules & Diseases Mar 2022Progressive pancytopenia is a common feature observed in DNA crosslink repair deficiency disorder, Fanconi anemia (FA). However, this phenotype has not been...
Progressive pancytopenia is a common feature observed in DNA crosslink repair deficiency disorder, Fanconi anemia (FA). However, this phenotype has not been recapitulated in single FA gene knockout animal models. In this study, we analyzed hematological characteristics in zebrafish null mutants for two FA genes, fanca and fanco. In adult mutants, we demonstrate age-associated reduction in blood cell counts for all lineages, resembling progressive pancytopenia in FA patients. In larval mutants, we demonstrate vascular injury-induced thrombosis defects, particularly upon treatment with crosslinking agent diepoxybutane (DEB), indicating DNA damage induced inefficiency of thrombocytes.
Topics: Animals; DNA Damage; Fanconi Anemia; Humans; Pancytopenia; Thrombosis; Zebrafish
PubMed: 34991062
DOI: 10.1016/j.bcmd.2021.102640 -
Internal and Emergency Medicine Aug 2021
Topics: Aged, 80 and over; Diagnosis, Differential; Dyspnea; Edema; Female; Fever; Humans; Leukemia, Mast-Cell; Pancytopenia; Positron-Emission Tomography; Tomography, X-Ray Computed
PubMed: 32382849
DOI: 10.1007/s11739-020-02333-z -
SN Comprehensive Clinical Medicine 2022Pancytopenia is a condition when a person has a low count of all three types of blood cells, causing a triage of anaemia, leukopenia and thrombocytopenia. It should not...
Pancytopenia is a condition when a person has a low count of all three types of blood cells, causing a triage of anaemia, leukopenia and thrombocytopenia. It should not be considered a disease in itself but rather a sign of a disease that needs to be further evaluated. Among the various causes, viral infections like the human immunodeficiency virus, cytomegalovirus, Epstein-Barr virus and parvovirus B19 have been implicated. Pancytopenia is a rare complication and is not commonly seen in patients with COVID-19 disease. Here, we report a case of pancytopenia in a previously immunocompetent elderly male patient with SARS-CoV-2 infection.
PubMed: 35036849
DOI: 10.1007/s42399-021-01085-x