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BMJ Case Reports Aug 2023Pancytopenia due to systemic lupus erythematosus (SLE) is rarely reported, and among those reported, it is mostly due to immunologically mediated cell destruction....
Pancytopenia due to systemic lupus erythematosus (SLE) is rarely reported, and among those reported, it is mostly due to immunologically mediated cell destruction. Pancytopenia due to bone marrow fibrosis secondary to SLE is an extremely rare entity. Myelofibrosis secondary to SLE per se is reported only in 21 cases in the literature. Ours probably is the 22nd case report on SLE with myelofibrosis. Primary presentation of SLE with bleeding manifestation is also a rare phenomenon. Partial to complete regression of myelofibrosis is noted following treatment in secondary myelofibrosis caused by SLE. We report a case of a woman in her late 40s who presented to us with bleeding manifestations of petechial rash and menorrhagia, which on further evaluation showed pancytopenia due to myelofibrosis secondary to SLE. Our case underlines multiple features like primary bleeding manifestation and regression of myelofibrosis following treatment which is rarely reported in association with SLE.
Topics: Female; Humans; Primary Myelofibrosis; Pancytopenia; Lupus Erythematosus, Systemic; Menorrhagia
PubMed: 37591626
DOI: 10.1136/bcr-2023-255229 -
International Journal of Laboratory... Oct 2022
Topics: Humans; Mycoses; Pancytopenia; Talaromyces
PubMed: 35244336
DOI: 10.1111/ijlh.13822 -
Pediatrics in Review Dec 2022
Topics: Male; Humans; Infant; Pancytopenia; Fever; Transaminases
PubMed: 36450638
DOI: 10.1542/pir.2021-004969 -
The Canadian Veterinary Journal = La... Dec 2021A 4-year-old neutered male St. Bernard-mastiff crossbred dog showed clinical signs of lethargy and anorexia after being administered phenobarbital for the treatment of...
A 4-year-old neutered male St. Bernard-mastiff crossbred dog showed clinical signs of lethargy and anorexia after being administered phenobarbital for the treatment of idiosyncratic seizures. A complete blood (cell) count revealed pancytopenia. Auto-agglutination and Coombs tests were negative suggesting that an immunemediated cause was unlikely; therefore, an idiosyncratic reaction to phenobarbital was suspected. Supportive care and control of seizures with zonisamide was initiated and clinical signs improved. Blood values were monitored closely and returned to normal after 3 wk.
Topics: Animals; Dog Diseases; Dogs; Male; Pancytopenia; Phenobarbital; Seizures
PubMed: 34857972
DOI: No ID Found -
Annals of Hematology Mar 2015Pancytopenia is a very rare complication of thymoma and has been sporadically reported in only a few cases. We report a case of a 68-year-old woman who presented with... (Review)
Review
Pancytopenia is a very rare complication of thymoma and has been sporadically reported in only a few cases. We report a case of a 68-year-old woman who presented with pancytopenia associated with thymoma. After failing high-dose corticosteroids, she responded to cyclosporine treatment and underwent successful thymectomy. We also reviewed all other similar cases published in the English language literature. Surgical resection by itself was generally ineffective for treatment of pancytopenia, and immunosuppressive therapy was required for bone marrow recovery. Resolution of pancytopenia was most frequently associated with cyclosporine-based therapy with a response rate (RR) of 66.6 %. In conclusion, pancytopenia associated with thymoma requires medical treatment, and the evidence presented here suggests that a cyclosporine-based regimen should be considered for initial therapy.
Topics: Aged; Disease-Free Survival; Female; Humans; Immunosuppressive Agents; Pancytopenia; Remission Induction; Standard of Care; Thymoma; Thymus Neoplasms
PubMed: 25315166
DOI: 10.1007/s00277-014-2230-x -
Blood Mar 2023Acute graft-versus-host disease (GVHD) is a rare complication after solid organ transplantation (SOT) that carries high mortality. Caused by immunocompetent donor...
Acute graft-versus-host disease (GVHD) is a rare complication after solid organ transplantation (SOT) that carries high mortality. Caused by immunocompetent donor leukocytes within the transplanted organ, which become activated against recipient tissues, GVHD typically develops 2 to 12 weeks after SOT and can affect the skin, gastrointestinal tract, liver, and bone marrow. Signs and symptoms are nonspecific and include a rash, nausea, appetite loss, diarrhea, and cytopenias. Pancytopenia from marrow-directed GVHD is the primary driver of mortality. The diagnosis of GVHD is often delayed but should be confirmed by biopsy of an affected organ. Evidence of donor chimerism in blood or marrow supports the diagnosis. When GVHD is diagnosed we initiate treatment with systemic corticosteroids. At that time, if GVHD only involves skin or oral mucosa we also decrease maintenance immunosuppression levels to allow the recipient to reject the donor immune cells. For GVHD involving the marrow we initiate an allogeneic hematopoietic cell donor search early. In this article, we describe 3 cases of GVHD after SOT, outline our approach to diagnosis and management, and then provide analysis of the 3 instructive cases.
Topics: Humans; Graft vs Host Disease; Organ Transplantation; Bone Marrow; Pancytopenia; Adrenal Cortex Hormones; Bone Marrow Transplantation
PubMed: 36395067
DOI: 10.1182/blood.2022015954 -
NeoReviews Dec 2021
Topics: Humans; Ichthyosis; Infant; Infant, Newborn; Infant, Premature; Infant, Very Low Birth Weight; Pancytopenia; Sepsis
PubMed: 34850146
DOI: 10.1542/neo.22-12-e843 -
Transfusion and Apheresis Science :... Aug 2021Autoimmune hemolytic anemia (AIHA), immune thrombocytopenia (ITP), and autoimmune neutropenia (AIN) are reported in the literature after liver, intestinal, heart,...
INTRODUCTION
Autoimmune hemolytic anemia (AIHA), immune thrombocytopenia (ITP), and autoimmune neutropenia (AIN) are reported in the literature after liver, intestinal, heart, pancreas, and kidney transplants. We report a case of autoimmune pancytopenia (AIHA, AIN and ITP) 9 years after liver transplantation with confirmed erythrocyte and neutrophil auto-antibodies.
CASE REPORT
A 49 years old man was admitted to our hospital presented with dysentery and fever, with history of liver transplantation in 2008. Laboratory evaluation demonstrated hemoglobin: 7.2 g/dL, granulocytes: 0.10 × 10/L and platelets: 15 × 10/mm³; indirect bilirubin: 3.62 mg/dL; lactate dehydrogenase: 603 U/L. Direct antiglobulin test revealed a monospecific anti-IgG plus C3 and the acid eluate was reactive to all panel red cells, consistent with an AIHA. Granulocyte immunofluorescence test (GIFT) and agglutination test (GAT) were reactive for granulocytes. Test with Luminex technology for human neutrophil antigen (HNA) antibody detection was strong reactive with beads expressing HNA-1a, -1b, -1c, -2, -4a and -5a antigens. HNA genotyping revealed the presence of the corresponding antigens, confirming the autoantibodies. Test with Luminex technology for human leucocyte antigen (HLA) antibody detection was negative. Monoclonal antibody immobilization of platelet antigens (MAIPA) assay was negative. Viral causes were excluded. The condition was compatible with clinical onset of autoimmune pancytopenia. Prednisone was administered at an initial dose of 1 mg/kg/day and immunosuppressive therapy was adjusted. This treatment resulted in rapid resolution of pancytopenia.
CONCLUSION
Combined autoimmune pancytopenia (AIHA, AIN and ITP) is a rare condition that may occur after liver transplantation. Early recognition of this phenomenon permits appropriate treatment.
Topics: Autoantibodies; Autoimmune Diseases; Humans; Immunosuppression Therapy; Liver Transplantation; Male; Middle Aged; Pancytopenia; Prednisolone
PubMed: 33895070
DOI: 10.1016/j.transci.2021.103136 -
BMC Pediatrics Aug 2023Lysinuric protein intolerance is a rare inherited metabolic disease due to autosomal recessive mutations of the SLC7A7 gene. The affected patients commonly present with...
BACKGROUND
Lysinuric protein intolerance is a rare inherited metabolic disease due to autosomal recessive mutations of the SLC7A7 gene. The affected patients commonly present with protein-rich food intolerance, failure to thrive, hepatosplenomegaly, muscle hypotonia and lung involvement due to impaired intestinal absorption and excessive urinary excretion of dibasic amino acids. Presentation with splenomegaly and cytopenia without other features has not been reported. Here we report a Sri Lankan girl with lysinuric protein intolerance presenting with pancytopenia and splenomegaly mimicking acute leukaemia.
CASE PRESENTATION
Two years and six months old Sri Lankan girl presented with persistent pancytopenia following a viral illness. She was asymptomatic without vomiting, diarrhoea, abdominal pain or irritability. Physical examination revealed pallor and isolated firm splenomegaly of 2 cm. Growth parameters and other system examinations were normal. Full blood count revealed anaemia, leukopenia and thrombocytopenia. The blood picture showed a mixture of hypochromic microcytic and normochromic normocytic red cells with occasional pencil cells and macrocytes. Bone marrow examination was normal except for occasional megaloblasts; however, serum vitamin B12 and red blood cell folate were normal. The metabolic screen showed a high anion gap compensated metabolic acidosis, high lactate and ketosis. Genetic mutation analysis using whole exome sequencing revealed compound heterozygous variants of the SLC7A7 gene, confirming the diagnosis of lysinuric protein intolerance.
CONCLUSION
We report a child with lysinuric protein intolerance presenting with pancytopenia and splenomegaly without other disease features. This case report adds to the heterogenic presentations of lysinuric protein intolerance, which is considered a multifaceted disease.
Topics: Child; Female; Humans; Infant; Pancytopenia; Splenomegaly; Amino Acid Metabolism, Inborn Errors; Leukemia; Leukopenia; Thrombocytopenia; Amino Acid Transport System y+L
PubMed: 37528333
DOI: 10.1186/s12887-023-04207-7 -
JAMA Dermatology Jul 2023
Topics: Humans; Multiple Myeloma; Pancytopenia; Neoplasm Recurrence, Local; Lenalidomide; Thalidomide; Erythema; Dexamethasone; Antineoplastic Combined Chemotherapy Protocols
PubMed: 37285118
DOI: 10.1001/jamadermatol.2023.1450