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1.
BMJ Case Rep ; 14(8)2021 Aug 05.
Artigo em Inglês | MEDLINE | ID: mdl-34353830

RESUMO

A 40-year-old woman presented to our emergency department in an altered state following a generalised tonic-clonic seizure. On regaining consciousness, she gave a history of bleeding tendencies and menorrhagia, fatigue, nausea, vomiting and appetite loss for a long time. She had received multiple blood transfusions in the last 10 years. Investigations revealed severe hyponatraemia, transaminitis and pancytopenia, which showed cyclical fluctuations in the hospital. Hyponatraemia was attributed to a central cause owing to secondary hypothyroidism and hypocortisolism on evaluation. A diagnosis of cyclical thrombocytopenia was made by logging the trends of blood cell lines and applying the Lomb-Scargle test. Liver biopsy showed features of transfusion hemosiderosis explaining transaminitis. All of the haematological abnormalities and clinical symptoms resolved on thyroxine and corticosteroid replacement, suggesting causal association hypopituitarism with cyclical thrombocytopenia.


Assuntos
Insuficiência Adrenal , Hiponatremia , Hipopituitarismo , Pancitopenia , Adulto , Feminino , Humanos , Hiponatremia/diagnóstico , Hiponatremia/etiologia , Hipopituitarismo/complicações , Hipopituitarismo/diagnóstico , Hipopituitarismo/tratamento farmacológico , Convulsões
2.
Artigo em Alemão | MEDLINE | ID: mdl-34425609

RESUMO

Between April 2018 and August 2019, 6 dogs with laboratory diagnostic evidence of Fanconi syndrome were presented to the Tierklinik Hofheim. The presumptive diagnosis was confirmed via urine amino acid analysis in all dogs. In 5 of the 6 dogs an alimentary origin was suspected, in 3 dogs the course of the disease could be followed. Supportive therapeutic measures and elimination of jerky treats from the dogs' diet improved the clinical symptoms in all dogs with known follow-up. This is the first case series of dogs with alimentary acquired Fanconi syndrome in Germany. The frequency of occurrence of the disease in that short period of time in only one small animal clinic suggests a significantly higher incidence than currently assumed.


Assuntos
Doenças do Cão , Síndrome de Fanconi , Pancitopenia , Animais , Dieta , Doenças do Cão/diagnóstico , Doenças do Cão/etiologia , Doenças do Cão/terapia , Cães , Síndrome de Fanconi/diagnóstico , Síndrome de Fanconi/etiologia , Síndrome de Fanconi/terapia , Síndrome de Fanconi/veterinária , Alemanha , Pancitopenia/veterinária
4.
Vnitr Lek ; 67(E-4): 26-28, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34275316

RESUMO

The authors present a case report of 59-years-old woman examined for pancytopenia recently diagnosed during hospitalization for bilateral interstitial pneumonia without any confirmed etiological agents. Concomitantly, some systemic symptoms like lack of appetite and weight loss were present. Primary hematological disease was ruled out. Positivity of serological screening for HIV-1,2 was rather surprising. Absolute count of CD4+ lymphocytes was 8/μl. Thus, HIV infection was already in stage of AIDS and retrospectively, the interstitial pneumonia has to be judged as AIDS-indicative illness.


Assuntos
Síndrome de Imunodeficiência Adquirida , Infecções por HIV , Pancitopenia , Síndrome de Imunodeficiência Adquirida/complicações , Linfócitos T CD4-Positivos , Feminino , Infecções por HIV/complicações , Humanos , Pessoa de Meia-Idade , Pancitopenia/etiologia , Estudos Retrospectivos
6.
Int J Lab Hematol ; 43 Suppl 1: 82-85, 2021 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-34288451

RESUMO

Unexplained blood cytopenias, in particular anemia, are often found in older individuals. The relationship between these cytopenias and myeloid neoplasms like myelodysplastic syndromes is currently poorly defined. Terminology used to describe patients with unexplained cytopenias and with clonally restricted hematopoiesis can be confusing and is evolving. This review uses a complex clinical case with borderline morphology and somatic mutations with high variant allele frequencies to illustrate a diagnostic approach to clonal cytopenias, and differentiation from myeloid neoplasms with a focus on appropriate ancillary testing. Testing for somatic mutations and variant allele frequency is helpful in assessing risk for progression to myeloid malignancy. The interpretation of mutation profiles in patients with cytopenia has been challenging, as some of these genes are commonly detected in elderly adults showing a normal blood count as well as in individuals with nonmalignant bone marrow failure syndromes. For patients with unexplained cytopenias, longitudinal follow-up including monitoring of blood counts may also be appropriate.


Assuntos
Hematopoiese Clonal , Pancitopenia/diagnóstico , Pancitopenia/etiologia , Idoso , Biomarcadores , Medula Óssea/patologia , Hematopoiese Clonal/genética , Diagnóstico Diferencial , Gerenciamento Clínico , Suscetibilidade a Doenças , Testes Hematológicos , Humanos , Imunofenotipagem , Masculino
7.
Blood ; 138(3): 221-233, 2021 07 22.
Artigo em Inglês | MEDLINE | ID: mdl-34292326

RESUMO

The Chd8 gene encodes a member of the chromodomain helicase DNA-binding (CHD) family of SNF2H-like adenosine triphosphate (ATP)-dependent chromatin remodeler, the mutations of which define a subtype of autism spectrum disorders. Increasing evidence from recent studies indicates that ATP-dependent chromatin-remodeling genes are involved in the control of crucial gene-expression programs in hematopoietic stem/progenitor cell (HSPC) regulation. In this study, we identified CHD8 as a specific and essential regulator of normal hematopoiesis. Loss of Chd8 leads to severe anemia, pancytopenia, bone marrow failure, and engraftment failure related to a drastic depletion of HSPCs. CHD8 forms a complex with ATM and its deficiency increases chromatin accessibility and drives genomic instability in HSPCs causing an activation of ATM kinase that further stabilizes P53 protein by phosphorylation and leads to increased HSPC apoptosis. Deletion of P53 rescues the apoptotic defects of HSPCs and restores overall hematopoiesis in Chd8-/- mice. Our findings demonstrate that chromatin organization by CHD8 is uniquely necessary for the maintenance of hematopoiesis by integrating the ATM-P53-mediated survival of HSPCs.


Assuntos
Proteínas de Ligação a DNA/metabolismo , Hematopoese , Células-Tronco Hematopoéticas/citologia , Proteína Supressora de Tumor p53/metabolismo , Animais , Apoptose , Proteínas Mutadas de Ataxia Telangiectasia/metabolismo , Sobrevivência Celular , Células Cultivadas , Proteínas de Ligação a DNA/genética , Deleção de Genes , Células-Tronco Hematopoéticas/metabolismo , Camundongos , Pancitopenia/genética , Pancitopenia/metabolismo , Estabilidade Proteica
8.
J Investig Med High Impact Case Rep ; 9: 23247096211029744, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34229452

RESUMO

We report a rare case of a 32-year-old male who ingested 32.4 to 54 mg of colchicine and presented after 44 hours. He developed progressive multiple organ failure with shock, acute kidney failure, troponemia, pancytopenia, absolute neutropenia, disseminated intravascular coagulation, acute liver failure, rhabdomyolysis, and lactic acidosis. He also developed electrolyte abnormalities and refractory hypoglycemia. Initial treatment consisted of activated charcoal, fluids, and broad-spectrum antibiotics with supportive treatment of mechanical ventilation, hemodialysis, vasopressors, N-acetylcysteine, colony-stimulating factors, and blood products. Literature shows potential benefit of colchicine-specific Fab fragments for acute toxicity with limited studies and is not currently available in the United States. Further research for N-acetylcysteine protocol for acute liver failure in colchicine toxicity and potential use of colchicine-specific Fab fragments is needed. Our case demonstrates the importance of early use of activated charcoal for ingestion overdose with the incorporation of poison control into multidisciplinary team for coordinated patient care.


Assuntos
Injúria Renal Aguda , Overdose de Drogas , Pancitopenia , Adulto , Colchicina/efeitos adversos , Humanos , Masculino , Insuficiência de Múltiplos Órgãos
11.
Int J Mol Sci ; 22(11)2021 May 21.
Artigo em Inglês | MEDLINE | ID: mdl-34064196

RESUMO

BACKGROUND: Chimeric antigen receptor (CAR) T-cells are changing the therapeutic landscape of hematologic malignancies. Severe side effects include cytokine release syndrome (CRS) and immune effector cell-associated neurotoxicity syndrome (ICANS), but prolonged cytopenia has also been reported. The underlying mechanism for prolonged cytopenia is poorly understood so far. CASES: Severe pancytopenia with grade 2-3 anemia was marked 2-3 months after treatment. Laboratory evaluation revealed undetectable levels of haptoglobin with increased reticulocyte counts. Coomb's tests were negative, no schistocytes were detected on blood smear, and infectious causes were ruled out. Increased erythropoiesis without lymphoma infiltration was noted on bone marrow biopsy. A spontaneous increase in haptoglobin and hemoglobin levels was observed after several weeks. For one patient, peripheral CAR-T levels were monitored over time. We observed a decline at the same time as hemoglobin levels began to rise, implying a potential causality. CONCLUSION: To our knowledge, we describe the first two cases of Coombs-negative hemolytic anemia after CAR-T treatment for B-cell lymphoma. We encourage routine monitoring for hemolytic anemia after CAR-T treatment and also encourage further investigations on the underlying mechanism.


Assuntos
Anemia Hemolítica/etiologia , Terapia Baseada em Transplante de Células e Tecidos/efeitos adversos , Pancitopenia/etiologia , Receptores de Antígenos Quiméricos/imunologia , Feminino , Neoplasias Hematológicas/imunologia , Neoplasias Hematológicas/terapia , Humanos , Linfoma/genética , Masculino , Pessoa de Meia-Idade
13.
Radiat Res ; 196(3): 297-305, 2021 09 01.
Artigo em Inglês | MEDLINE | ID: mdl-34129665

RESUMO

Survival from partial-body irradiation (PBI) may be limited by the development of the late lung injury response of pneumonitis. Herein we investigated the hypothesis that acute hematopoietic depletion alters the onset and severity of lung disease in a mouse model. To establish depletion, C3H/HeJ mice received 8 Gy PBI with shielding of only the tibiae, ankles and feet. One week after irradiation, blood lymphocyte and neutrophil counts were each significantly reduced (P < 0.04) in these mice compared to levels in untreated controls or in mice receiving 16 Gy to the whole thorax only. All 8 Gy PBI mice survived to the experimental end point of 16 weeks postirradiation. To determine whether the hematopoietic depletion affects lung disease, groups of mice received 8 Gy PBI plus 8 Gy whole-thorax irradiation (total lung dose of 16 Gy) or 16 Gy whole-thorax irradiation only. The weight loss, survival to onset of respiratory distress (P = 0.17) and pneumonitis score (P = 0.96) of mice that received 8 Gy PBI plus 8 Gy whole-thorax irradiation were not significantly different from those of mice receiving 16 Gy whole-thorax irradiation only. Mice in respiratory distress from PBI plus whole-thorax irradiation had significantly reduced (P = 0.02) blood monocyte counts compared to levels in distressed, whole-thorax irradiated mice, and symptomatic pneumonitis was associated with increased blood neutrophil counts (P = 0.04) relative to measures from irradiated, non-distressed mice. In conclusion, survivable acute hematopoietic depletion by partial-body irradiation did not alter the onset or severity of lethal pneumonitis in the C3H/HeJ mouse model.


Assuntos
Pancitopenia/etiologia , Lesões Experimentais por Radiação/terapia , Pneumonite por Radiação/prevenção & controle , Animais , Progressão da Doença , Feminino , Inflamação/prevenção & controle , Contagem de Leucócitos , Pulmão/patologia , Pulmão/efeitos da radiação , Camundongos , Camundongos Endogâmicos C3H , Lesões Experimentais por Radiação/sangue , Lesões Experimentais por Radiação/etiologia , Pneumonite por Radiação/sangue , Pneumonite por Radiação/etiologia , Pneumonite por Radiação/patologia , Tórax/efeitos da radiação , Perda de Peso/efeitos da radiação
14.
J Med Case Rep ; 15(1): 247, 2021 May 19.
Artigo em Inglês | MEDLINE | ID: mdl-34006332

RESUMO

BACKGROUND: Prostate adenocarcinoma is the most frequent cancer type among men, followed by skin cancer. Patients with prostate cancer usually present lower urinary tract symptoms due to tumor involvement. Bone marrow invasion is associated with prostate cancer metastasis and is common if blastic lesions in bones are present but is very rare without a large bone involvement and uncommon as initial presentation. CASE PRESENTATION: We present a case of an 86-year-old Caucasian man with bone marrow invasion of prostate cancer without urological or bone-related symptoms and without prostate nodules. His findings were dyspnea, fatigue, and tachycardia. We detail the complete investigation of the case until we found the accurate diagnosis. The patient started treatment, but he had no response and so the oncology team started palliative care. CONCLUSION: Bone marrow invasion as an initial manifestation of prostate cancer is not common, especially if no prostatic lesions are found. This report is important to provide additional information about prostate cancer management.


Assuntos
Pancitopenia , Neoplasias da Próstata , Neoplasias Cutâneas , Idoso de 80 Anos ou mais , Humanos , Masculino , Pancitopenia/etiologia , Antígeno Prostático Específico , Neoplasias da Próstata/complicações
18.
BMJ Case Rep ; 14(3)2021 Mar 31.
Artigo em Inglês | MEDLINE | ID: mdl-33789863

RESUMO

Haemophagocytic lymphohistiocytosis (HLH) is a rare diagnosis that carries a high degree of mortality. We present this case of a previously healthy 22-year-old woman, who was admitted acutely ill to the hospital. One week prior, she had been seen by her primary care physician for fatigue and malaise. At that time, she was noted to have anterior and posterior cervical lymphadenopathy. She was referred to the emergency room and was diagnosed with acute Epstein-Barr virus (EBV) mononucleosis based on her clinical symptoms and positive heterophile antibody test. She was discharged after an uneventful 48-hour stay on the wards. She represented 7 days after discharge with cough, fatigue, nausea, vomiting, epigastric abdominal pain, diarrhoea, weight loss and subjective fevers. She had also reported haematemesis, epistaxis and melaena. Vital signs included temperature 36.9°C, blood pressure 90/50 mm Hg, heart rate 130 beats per minute and respiratory rate 32 breaths per minute. Physical examination was notable for an acutely ill appearing woman with scleral icterus, hepatosplenomegaly and palpable cervical and axillary lymphadenopathy. Complete blood count showed pancytopaenia with haemoglobin 59 g/L (normal 120-160 g/L), white blood cell count 2.7×109/L (normal 4-10.5×109/L) and platelet count 50×109/L (normal 150-450×109/L). The white blood cell count differential included 58% neutrophils (normal 38%-77%) with immature neutrophils in band form elevated at 45% (normal <14%), 16% lymphocytes (normal 20%-48%), 7% monocytes (normal <12%) and no eosinophils (normal <6%). Blood smear revealed anisocytosis, poikilocytosis and hypochromia. Coagulation panel showed elevated levels of d-dimer level at 1.39 µg/mL (normal <0.45 µg/mL), prolonged prothrombin time at 34.4 s (normal 11-15 s), prolonged activated partial thromboplastin time of 55.6 s (normal 25-34 s), prolonged international normalised ratio at 3.31 (normal <1.1) and low fibrinogen 60 mg/dL (normal >200 mg/dL). Lipid panel showed cholesterol at 114 mg/dL (normal 125-200 mg/dL), triglycerides 207 mg/dL (normal 30-150 mg/dL), high-density lipoprotein cholesterol 10 mg/dL (normal 40-60 mg/dL) and low-density lipoprotein cholesterol 63 mg/dL (normal <100 mg/dL). Other lab abnormalities included elevated ferritin of 6513 ng/mL (normal 10-150 ng/mL) and elevated lactate dehydrogenase of 1071 unit/L (normal 95-240 unit/L). Soluble interleukin-2 receptor alpha level was elevated at 60 727 units/mL (normal 223-710 units/mL). Fluorodeoxyglucose-positron emission tomography (FDG-PET) scan showed abnormal tracer localisation within the paratracheal, hilar, pelvic, abdominal and subcarinal lymph nodes, along with FDG-PET positive hepatosplenomegaly. A bone marrow biopsy showed hypercellular marrow (95% cellularity) with trilineage haematopoiesis, haemophagocytic cells, polytypic plasmacytosis and T-cell lymphocytosis, along with positive latent membrane protein-1 immunohistochemical staining for EBV. EBV quantitative DNA PCR showed >1 million copies. These findings were consistent with a diagnosis of HLH secondary to EBV infection. Despite intense therapy with the HLH-94 protocol, the patient expired from her illness after a prolonged hospital course.


Assuntos
Infecções por Vírus Epstein-Barr , Mononucleose Infecciosa , Linfo-Histiocitose Hemofagocítica , Pancitopenia , Adulto , Infecções por Vírus Epstein-Barr/complicações , Infecções por Vírus Epstein-Barr/diagnóstico , Feminino , Herpesvirus Humano 4 , Humanos , Linfo-Histiocitose Hemofagocítica/complicações , Linfo-Histiocitose Hemofagocítica/diagnóstico , Adulto Jovem
19.
Clin J Gastroenterol ; 14(3): 820-826, 2021 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-33886104

RESUMO

Idiopathic portal hypertension (IPH) is one of the background diseases causing nodular regenerative hyperplasia (NRH). Furthermore, IPH patients accompanied with autoimmune diseases, such as systemic lupus erythematosus (SLE) and systemic sclerosis (SSc), are more likely to form NRH in the liver. A 76-year-old woman had been aware of the Raynaud's phenomenon and scleroderma for the past 30 years. In this case, she presented with abdominal fullness, and her imaging analysis revealed ascites and multiple liver nodules. On Gd-EOB-DTPA enhanced magnetic resonance imaging (EOB-MRI), donut-like uptake was observed in the nodules in the hepatobiliary phase. Liver biopsy of a nodule demonstrated that it was composed of hyperplastic hepatocytes without fibrous septa, and dilated sinusoids were observed beside the nodule. Conversely, background liver showed that peripheral portal veins appeared stenotic with dense fibrosis in the portal area. The final diagnosis was that multiple NRH of the liver developed in SSc patient accompanying IPH. This case suggests that NRH may be unexpectedly diagnosed in patients with autoimmune diseases accompanying IPH.


Assuntos
Hipertensão Portal , Escleroderma Sistêmico , Idoso , Feminino , Humanos , Hiperplasia/patologia , Hipertensão Portal/complicações , Fígado/diagnóstico por imagem , Fígado/patologia , Cirrose Hepática/patologia , Pancitopenia , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/patologia , Esplenomegalia
20.
Rev Bras Enferm ; 74(1): e20200644, 2021.
Artigo em Inglês, Português | MEDLINE | ID: mdl-33886929

RESUMO

OBJECTIVES: to assess/correlate health-related quality of life with the social dimension of hematopoietic, autologous and allogeneic stem cell transplant patients in the three years post-transplant. METHODS: longitudinal, observational study with 55 patients, in a reference hospital in Latin America, from September 2013 to February 2019, using the Quality of Life Questionnaire-Core and Functional Assessment Cancer Therapy Bone Marrow Transplantation. RESULTS: A total of 71% underwent allogeneic transplantation. The social dimension had low averages since the baseline stage (55, 21) and low scores (56) for quality of life in pancytopenia. There was a significant positive correlation between social dimension, quality of life in pancytopenia (p<0.01) and follow-up after hospital discharge (p<0.00). There is a significant difference (p<0.00) throughout the stages, however, not in terms of the type of transplant (p>0.36/0.86). CONCLUSIONS: patients with better assessments in the social dimension have a better quality of life. Interventions focusing on the multidimensionality of the quality of life construct are necessary.


Assuntos
Transplante de Células-Tronco Hematopoéticas/psicologia , Pancitopenia/cirurgia , Qualidade de Vida , Isolamento Social , Adulto , Idoso , Transplante de Medula Óssea , Feminino , Humanos , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Enfermagem Oncológica , Inquéritos e Questionários , Adulto Jovem
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