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1.
Rev. baiana saúde pública ; 45(3,supl.n.esp): 89-97, 28 dec. 2021.
Article in Portuguese | LILACS | ID: biblio-1352333

ABSTRACT

Alterações hematológicas, como anemia ou pancitopenia, podem ser manifestações atípicas do hipertireoidismo. Embora a maioria dos casos de pancitopenia na doença de Graves seja decorrente do uso de drogas antitireoidianas ­ tionamidas ­, ela também pode estar associada ao quadro de hipertireoidismo não tratado e, assim, se manifestar como uma complicação grave. Este estudo tem como objetivo relatar o caso de uma paciente portadora de hipertireoidismo que evoluiu durante o internamento por pancitopenia secundária à doença primária. As informações foram obtidas por meio da revisão do prontuário, entrevista com a paciente e revisão da literatura. Trata-se de uma paciente do sexo feminino, de 39 anos, internada em um hospital de Salvador por apresentar um quadro de edema em membros inferiores que evoluiu progressivamente para anasarca por 45 dias. Associados ao quadro, a paciente apresentava perda ponderal, dispneia paroxística noturna, taquicardia, sudorese e calafrios, além dos exames laboratoriais terem apresentado pancitopenia. O caso relatado demonstra uma manifestação atípica e rara do hipertireoidismo, que melhorou após o início do tratamento e do estado de eutireoidismo.


Hematological changes such as anemia or pancytopenia can occur as atypical manifestations of hyperthyroidism. Although most cases of pancytopenia in Graves' disease are due to the use of antithyroid drugs (thionamides), it can also be associated with untreated hyperthyroidism, thus manifesting as a serious complication. This study aims to report the case of a 39-year-old hyperthyroid female patient who developed pancytopenia during hospitalization. Data were collected by means of the medical record, an interview conducted with the patient, and literature review. The patient was admitted to a hospital in Salvador presenting lower extremity edema that progressively evolved to anasarca within 45 days prior to the interview. She also presented weight loss, paroxysmal nocturnal dyspnea, tachycardia, sweating, chills, and laboratory test results for pancytopenia. The case reported here demonstrates an atypical and rare manifestation of hyperthyroidism that improved after the beginning of the treatment, reaching the euthyroid state.


Los cambios hematológicos como anemia o pancitopenia pueden ocurrir como manifestaciones atípicas de hipertiroidismo. Aunque la mayoría de los casos de pancitopenia en la enfermedad de Graves se deben al uso de fármacos antitiroideos (tionamidas), también se puede asociar a um hipertiroidismo no tratado y, por tanto, puede manifestarse como una complicación grave. Este estudio tiene como objetivo reportar el caso de una paciente con hipertiroidismo que desarrolló durante la hospitalización pancitopenia secundaria a la enfermedad primaria. La información se obtuvo revisando la historia clínica, entrevistando a la paciente y revisando la literatura. Se trata de una paciente de 39 años ingresada en un hospital de Salvador por presentar edema en miembros inferiores, evolucionando progresivamente a anasarca durante 45 días. Asociado a la afección, presentaba pérdida de peso, disnea paroxística nocturna, taquicardia, sudoración, escalofríos y análisis de laboratorio que mostraban pancitopenia. El caso aquí reportado demuestra una manifestación atípica y rara de hipertiroidismo, que evolucionó luego del inicio del tratamiento y del estado de eutiroidismo.


Subject(s)
Humans , Female , Pancytopenia , Bone Marrow , Hyperthyroidism
3.
Arch. argent. pediatr ; 118(2): e174-e177, abr. 2020.
Article in English, Spanish | LILACS, BINACIS | ID: biblio-1100429

ABSTRACT

La linfohistiocitosis hemofagocítica (LHH) puede ser primaria (hereditaria) o secundaria a infecciones, tumores malignos, trastornos reumatológicos, síndromes de inmunodeficiencia y metabolopatías. Se informaron casos de intolerancia a la proteína lisinúrica, deficiencia de múltiples sulfatasas, galactosemia, enfermedad de Gaucher, síndrome de Pearson y galactosialidosis. No se sabe cómo se desencadena la LHH en las metabolopatías. Se diagnosticó LHH en un lactante de 2 meses con letargo, palidez, alimentación deficiente, hepatoesplenomegalia, fiebre y pancitopenia, y se instauró el protocolo HLH-2004. Se realizaron, en conjunto, análisis para detectar mutaciones genéticas y pruebas metabólicas; los resultados fueron negativos para las mutaciones genéticas de LHH primaria, pero se detectaron hiperamoniemia y concentración elevada de metilcitrato. Se diagnosticó acidemia propiónica. Aquí informamos sobre un caso de LHH secundaria a acidemia propiónica. Es posible la realización simultánea de pruebas de detección de trastornos metabólicos y de mutaciones genéticas para el diagnóstico temprano en los lactantes con LHH


Hemophagocytic lymphohystiocytosis (HLH) may be primary (inherited/familial) or secondary to infections, malignancies, rheumatologic disorders, immune deficiency syndromes and metabolic diseases. Cases including lysinuric protein intolerance, multiple sulfatase deficiency, galactosemia, Gaucher disease, Pearson syndrome, and galactosialidosis have previously been reported. It is unclear how the metabolites trigger HLH in metabolic diseases. A 2-month-old infant with lethargy, pallor, poor feeding, hepatosplenomegaly, fever and pancytopenia, was diagnosed with HLH and the HLH-2004 treatment protocol was initiated. Analysis for primary HLH gene mutations and metabolic screening tests were performed together; primary HLH gene mutations were negative, but hyperammonemia and elevated methyl citrate were detected. Propionic acidemia was diagnosed with tandem mass spectrometry in neonatal dried blood spot. We report this case of HLH secondary to propionic acidemia. Both metabolic disorder screening tests and gene mutation analysis may be performed simultaneously especially for early diagnosis in infants presenting with HLH.


Subject(s)
Humans , Male , Infant , Lymphohistiocytosis, Hemophagocytic/diagnosis , Propionic Acidemia/diagnosis , Pancytopenia , Splenomegaly , Lymphohistiocytosis, Hemophagocytic/drug therapy , Propionic Acidemia/drug therapy , Torpor , Continuous Renal Replacement Therapy , Hepatomegaly
4.
Rev. Hosp. Ital. B. Aires (2004) ; 40(1): 25-28, mar. 2020. ilus
Article in Spanish | LILACS | ID: biblio-1102210

ABSTRACT

Introducción: la zigomicosis es una infección fúngica poco frecuente, con alta tasa de mortalidad y de mal pronóstico. Afecta principalmente a pacientes inmunocomprometidos. La asociación con el síndrome hemofagocítico es extremadamente inusual, más aún en pacientes inmunocompetentes, con pocos ejemplos registrados en la literatura. Caso clínico: se presenta el caso de un paciente masculino inmunocompetente de 40 años con diagnóstico de mucormicosis y síndrome hemofagocítico que evoluciona desfavorablemente, con fallo multiorgánico, a pesar de los esfuerzos médicos. Conclusión: la asociación de mucormicosis con síndrome hemofagocítico en un paciente inmunocompetente es extremadamente rara; existen pocos casos informados en Latinoamérica. Debemos tener presente esta asociación, ya que requiere un tratamiento agresivo y soporte vital avanzado. (AU)


Introduction: zygomycosis is a rare fungal infection that carries with high mortality rates. This poor prognosis, rapidly progressive infection mainly affects immunocompromised patients. The association with hemophagocytic lymphohistiocytosis is extremely unusual, even more in immunocompetent patients, with few cases reported. Case: we present the case of an immunocompetent male patient who was diagnosed with zygomycosis and hemophagocytic lymphohistiocytosis. Despite medical efforts he developed multiorganic failure. Conclusion: the association of mucormycosis with hemophagocytic lymphohistiocytosis in an immunocompetent patient is exceptional with few cases reported in Latin America. We must always suspect this association considering they require aggressive treatment and advanced life support. (AU)


Subject(s)
Humans , Male , Adult , Zygomycosis/diagnosis , Lymphohistiocytosis, Hemophagocytic/diagnosis , Pancytopenia/blood , Psychomotor Agitation , Vancomycin/therapeutic use , Norepinephrine/administration & dosage , Norepinephrine/therapeutic use , Amphotericin B/therapeutic use , Exophthalmos/diagnostic imaging , Immunocompromised Host/immunology , Colistin/therapeutic use , Amoxicillin-Potassium Clavulanate Combination/administration & dosage , Amoxicillin-Potassium Clavulanate Combination/therapeutic use , Zygomycosis/etiology , Zygomycosis/mortality , Zygomycosis/epidemiology , Delirium , Lymphohistiocytosis, Hemophagocytic/etiology , Lymphohistiocytosis, Hemophagocytic/mortality , Fever , Meropenem/therapeutic use , Immunocompetence/immunology , Jaundice , Mucormycosis/complications , Multiple Organ Failure/diagnosis
5.
Diagn. tratamento ; 25(1): 9-12, jan.-mar. 2020. tab
Article in Portuguese | LILACS | ID: biblio-1099816

ABSTRACT

Contexto: A pancitopenia é uma condição clínica grave caracterizada pela diminuição simultânea dos níveis séricos dos glóbulos vermelhos, brancos e plaquetários. A sua resolução só ocorre quando tratado o processo etiológico. Descrição do caso: Nesse estudo, relatou-se o caso de um paciente pediátrico, procedente de região carente, que foi trazido ao serviço de referência da região pela mãe com quadro progressivo de queda do estado geral, inapetência, diarreia, náuseas e vômitos. Esses sintomas haviam se iniciado cinco dias antes. No recordatório alimentar, a responsável relatou dieta pobre em alimentos de origem animal e vegetal, tendo, ainda, histórico de internação recente. Ao exame físico, apresentou-se hipocorado, com palidez cutânea, sinais vitais normais e sem demais alterações dignas de nota. Inicialmente, solicitaram-se exames complementares que mostraram pancitopenia associada a leucocitúria. Instituídos sintomáticos e antibioticoterapia de amplo espectro para determinado foco. No sexto dia de internação, o paciente evoluiu com quadro séptico, necessitando de transferência e terapia intensiva. Após estabilização do quadro infeccioso, o paciente retornou à enfermaria do serviço para continuidade do tratamento e investigação etiológica da pancitopenia. Por meio de exames complementares, foi possível identificar que a anemia normocítica e normocrômica se tratava de deficiência de vitamina B12 de apresentação atípica. Discussão: Diante de casos com apresentação atípica associada à pancitopenia, é necessário considerar a possibilidade de deficiência de micronutrientes, por se tratar de uma condição frequente em nosso país. Conclusão: O presente relato de caso pode ser utilizado como alerta a profissionais de saúde, para que considerem a deficiência de cianocobalamina como causa de pancitopenia, principalmente em regiões mais vulneráveis, visto que essa condição pode estar associada a quadros graves e que ameaçam a vida.


Subject(s)
Humans , Male , Infant , Pancytopenia , Vitamin B 12 , Child Health , Hematologic Diseases , Anemia, Megaloblastic
6.
J. bras. nefrol ; 41(3): 427-432, July-Sept. 2019. tab
Article in English | LILACS | ID: biblio-1040255

ABSTRACT

Abstract Methotrexate is an effective medication to control several diseases; however, it can be very toxic, being myelosuppression one of its main adverse effects, which increases in severity and frequency in patients with renal failure. We present the case of a 68-year-old man with chronic, end-stage renal disease associated with ANCA vasculitis, under treatment with peritoneal dialysis, who received the medication at a low dose, indicated by disease activity, which presented as a complication with severe pancytopenia with mucositis that improved with support measures and multiple-exchange peritoneal dialysis. We reviewed 20 cases published to date of pancytopenia associated with methotrexate in patients on dialysis and found high morbidity and mortality, which is why its use in this type of patient is not recommended. However, when this complication occurs, a therapeutic option could be the use of multiple-exchange peritoneal dialysis in addition to supportive therapy for drug-related toxicity, although it is recognized that studies are required to show the role of multiple-exchange peritoneal dialysis in the removal of this medication.


Resumo Apesar de sua toxicidade, o metotrexato é um medicamento eficaz no controle de várias doenças. A mielossupressão, um de seus principais efeitos adversos, aumenta em gravidade e frequência nos pacientes com insuficiência renal. Apresentamos o caso de um homem de 68 anos de idade com doença renal terminal relacionada à vasculite associada ao ANCA em diálise peritoneal, que recebeu a medicação em dose baixa em função da atividade da doença e que teve como complicação pancitopenia grave com mucosite, tratada com medidas de suporte e diálise peritoneal com múltiplas trocas. Revisamos 20 casos publicados até o presente momento sobre pancitopenia associada a metotrexato em pacientes em diálise. Foi identificada alta morbidade e mortalidade, razão pela qual seu uso nesse tipo de paciente não é recomendado. No entanto, quando esta complicação ocorre, uma opção terapêutica pode ser o uso de diálise peritoneal com múltiplas trocas, além da terapia de suporte para toxicidade medicamentosa. Maiores estudos são necessários para demonstrar o papel da diálise peritoneal com múltiplas trocas na remoção desse medicamento.


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , Vasculitis/drug therapy , Methotrexate/adverse effects , Methotrexate/therapeutic use , Peritoneal Dialysis/methods , Folic Acid Antagonists/adverse effects , Folic Acid Antagonists/therapeutic use , Kidney Failure, Chronic/therapy , Pancytopenia/etiology , Pancytopenia/therapy , Shock, Septic/etiology , Shock, Septic/drug therapy , Methotrexate/blood , Treatment Outcome , Mucositis/etiology , Mucositis/drug therapy , Folic Acid Antagonists/blood , Anti-Bacterial Agents/therapeutic use
8.
Article in English | WPRIM | ID: wpr-740157

ABSTRACT

Metastasis of rhabdomysarcoma to the breast is a very rare manifestation in adult males. Herein, we report a case of metastasis from embryonal rhabdomyosarcoma in the left hypothenar muscle that presented as a breast mass in a 38-year-old man, who four months later expired because of multiple bone metastases related to pancytopenia. We describe the various imaging findings, including mammograms, ultrasonography, computerized tomography (CT), positron emission tomography-computed tomography (PET-CT), and magnetic resonance imaging (MRI) of this rare disease. The various imaging findings of this lesion could be helpful for future diagnosis of male breast lesions.


Subject(s)
Adult , Breast , Diagnosis , Electrons , Humans , Magnetic Resonance Imaging , Male , Neoplasm Metastasis , Pancytopenia , Rare Diseases , Rhabdomyosarcoma , Rhabdomyosarcoma, Embryonal , Ultrasonography
9.
Laboratory Medicine Online ; : 249-253, 2019.
Article in Korean | WPRIM | ID: wpr-760509

ABSTRACT

A 22-year old female patient with systemic lupus erythematosus presenting microangiopathic hemolytic anemia was treated with therapeutic plasma exchange 23 times. The patient's condition and laboratory findings (aspartate aminotransferase, alanine aminotransferase, ferritin, total bilirubin, and lactate dehydrogenase) did not improve despite the initial 18 therapeutic plasma exchange treatments. Thrombotic thrombocytopenic purpura was ruled out due to normal ADAMTS-13 activity test result; hemophagocytic lymphohistiocytosis was diagnosed based on fever, splenomegaly, pancytopenia, hypertriglyceridemia, hyperferritinemia, and hemophagocytosis in bone marrow aspiration. The patient's condition improved rapidly upon treatment with a combination of immunosuppressants and cytotoxic agents, and more therapeutic plasma exchanges were performed five consecutive times with prolonged intervals in between. We observed that therapeutic plasma exchange treatment alone was not effective enough to treat hemophagocytic lymphohistiocytosis, unlike thrombotic thrombocytopenic purpura. Therefore, it is necessary to determine and start drug administration promptly in the treatment of hemophagocytic lymphohistiocytosis with thrombotic microangiopathy.


Subject(s)
Alanine Transaminase , Anemia, Hemolytic , Bilirubin , Bone Marrow , Cytotoxins , Female , Ferritins , Fever , Humans , Hypertriglyceridemia , Immunosuppressive Agents , Lactic Acid , Lupus Erythematosus, Systemic , Lymphohistiocytosis, Hemophagocytic , Pancytopenia , Plasma Exchange , Plasma , Purpura, Thrombotic Thrombocytopenic , Splenomegaly , Thrombotic Microangiopathies
10.
Laboratory Medicine Online ; : 103-106, 2019.
Article in English | WPRIM | ID: wpr-760482

ABSTRACT

Bone marrow metastasis of colon cancer is rare. Here, we report a 56-year-old female patient who presented with pancytopenia. She was diagnosed with colon cancer accompanied by lung and axial skeleton metastasis. The bone marrow study showed metastatic carcinoma. Immunohistochemical (IHC) staining with anti-cytokeratin 7 (CK7) and anti-cytokeratin 20 (CK20) antibodies showed that the bone marrow samples were negative for CK7 and positive for CK20, consistent with metastatic colon cancer. To the best of our knowledge, there has been only one other reported case of bone marrow metastasis of colon cancer as the primary diagnosis in an adult patient in Korea. Bone and bone marrow metastases of colon cancer are regarded as uncommon. However, for proper management, bone marrows should be promptly examined in patients with solid tumors when unexplained cytopenia is noted, even if the origin of the tumor is known to be rarely metastatic to bone marrow. In addition, the use of cytokeratin IHC staining is helpful for determining the origin of metastatic carcinoma.


Subject(s)
Adult , Antibodies , Bone Marrow , Colon , Colonic Neoplasms , Diagnosis , Female , Humans , Immunohistochemistry , Keratins , Korea , Lung , Middle Aged , Neoplasm Metastasis , Pancytopenia , Skeleton
11.
Article in English | WPRIM | ID: wpr-760226

ABSTRACT

PURPOSE: The major side effects of treatment with oxcarbazepine (OXC) are skin rash and hyponatremia. Hematologic side effects are reported rarely. The aim of this study was to investigate the rate and types of the hematologic side effects of OXC. METHODS: The medical records of 184 patients diagnosed with epilepsy or movement disorder and on OXC monotherapy, at the Department of Pediatrics of Inje University Sanggye Paik Hospital from July 2001 to July 2018, were retrospectively reviewed. RESULTS: Of the 184 patients, 10 (5.4%) developed leukopenia in addition to pancytopenia and 2 (1.0%) developed pancytopenia. Leukopenia developed in 11 days to 14 years after OXC administration and was more frequent in males than in females (male vs. female, 9 vs. 1; Fisher exact test, P0.05, t-test). CONCLUSION: OXC-induced leukopenia is not rare and may result in pancytopenia. Patients being treated with OXC should be regularly monitored for abnormal complete blood count profiles.


Subject(s)
Blood Cell Count , Epilepsy , Exanthema , Female , Humans , Hyponatremia , Leukopenia , Lost to Follow-Up , Male , Medical Records , Movement Disorders , Pancytopenia , Pediatrics , Retrospective Studies
12.
Prensa méd. argent ; 104(5): 240-243, jul2018.
Article in Spanish | LILACS, BINACIS | ID: biblio-1049296

ABSTRACT

El tratamiento de pacientes con artritis reumatoide (AR) debe ser temprano y agresivo para prevenir el daño articular y la discapacidad. Los fármacos no biológicos modificadoes de enfermedad, como el metotrexato, han sido utilizados par controlar la actividad de la enfermedad y para prevenir el daño de las articulaciones. Existen pacientes con AR resistentes al tratamiento con fármacos modificadores de la enfermedad y otros que no responden adecuadamente a la terapia con inhibidores de factores de necrosis tumoral. Nosotros describimos el caso de una paciente de sexo femenino de 77 años que se presentó al servicio de emergencias con fiebre, mucositis y mal estado general luego de recibir una dosis de abatacept. A su ingreso el laboratorio demostró: glóbulos blancos 500 cel/mm3, neutrófilos 150 cel/mm3, plaquetas 21000 cel/mm3, hematocrito 29%, VCM 81, LDH 314 UI/L, función renal y hepatograma normales. En el examen clínico se objetivaron ulceras y lesiones ampollares en mucosa yugal. El medulograma evidenció hipocelularidad, con disminución de las tres series. El informe anatomopatológico fue de médula ósea hipoplásica. Recibió tratamiento con factor estimulante de colonias de neutrófilos, ácido fólico y metilprednisona, con resolución del cuadro a los 3 días de instituido el tratamiento. Hasta nuestro conocimiento esta es la primera comunicación de pancitopenia asociada a abatacept es una paciente con artritis AR intolerante a metotrexato


Treatment of patient with rheumatoid arthritis (RA) should be early and aggressive to prevent joint injury and disability. Disease-modifying antirheumatic drugs (DMARDs) like methotrexate has been used as initial treatment toward the disease activity and to prevento joint damage. Some patients with RA are resistant to initial therapy with nonbiiologic DMARDs or TNF inhibitiors. We described a 77 years old women who presented to the emergency room with fever andoral lesions after been treated with abatacept. On examination patient appeared ill. She had oral ulcers. laboratory testing showed white cells count 500 cells per mm3, hematocrit 29 %, platelets count 21000 cells per mm3, LDH 314 U/l. Renal and liver functions were normal. Bone marrow showed decreased in the three cells lineages. Patient was treated with granulocyte colony-stimulating factor, folic acid, and prednisone. Patient improved her physical and laboratory features three days after admission. This case showed the rare association between pancytopenia and abatacept in a patient with RA


Subject(s)
Humans , Female , Aged , Pancytopenia/diagnosis , Arthritis, Rheumatoid/therapy , Methotrexate/adverse effects , Methotrexate/therapeutic use , Abatacept/therapeutic use
13.
Annales des sciences de la santé ; 1(2): 114-120, 2018. tab
Article in French | AIM, AIM | ID: biblio-1259350

ABSTRACT

Introduction : La pancytopénie est définie comme étant une baisse associée des lignées érythrocytaire, granulocytaire et plaquettaire. L'origine est centrale ou périphérique. Cliniquement elle est caractérisée par une pâleur, de la fièvre et un syndrome hémorragique. C'est la première étude spécifique consacrée à ce sujet. Matériels et Méthodes: Il s'agit d'une étude portant sur 285 dossiers de patients sur 14 années. La pancytopénie était définie en présence des critères suivants : un taux d'Hb < 11 g/dl ; un taux de globules blancs (GB) < 4000/mmᶾ avec un taux de PNN < 1500 /mmᶾ ; un taux de plaquettes < 150 000/mmᶾ.Les paramètres étudiés avaient porté sur l'âge, le sexe, les signes cliniques (Pâleur, asthénie, fièvre, syndrome hémorragique), les résultats des examens paracliniques : hémogramme, myélogramme, biopsie ostéo-médullaire, goutte épaisse, sérologie VIH, hémocultures et échographie. Les critères de non inclusion étaient les cas de bicytopénie et les pancytopénie chimio-induite. Résultats : La fréquence des pancytopénies était de 20,5 cas/an; le sex-ratio: 0,8. La population était très jeune avec 70% de moins de 45 ans. Les principales manifestations cliniques étaient: la pâleur (271 cas : 95,1%), asthénie (253 cas: 88,8%), le syndrome tumoral (143 cas: 50,1%), la fièvre (133 cas: 46,7%), le syndrome hémorragique (48 cas: 17%). Les étiologies étaient dominées par les carences vitaminiques (87 cas : 30,5%), l'hypersplénisme (67 cas : 23,5%), les infections (45 cas : 15,8%), les hémopathies malignes (19 cas : 6,7%). Conclusion: Les étiologies des pancytopénies sont dominées par les carences vitaminiques et l'hypersplénisme. L'enquête étiologique doit être essentiellement axée sur ces 2 causes après avoir éliminé un syndrome infectieux et une leucémie aigüe qui sont souvent des urgences


Subject(s)
Hypersplenism , Niger , Pancytopenia/etiology , Patients
14.
Article in English | WPRIM | ID: wpr-717637

ABSTRACT

Aplastic anemia may develop secondary to environmental exposure to entities such as chemicals, medical drugs, and infectious agents. Fatal complications from antiepileptic medications may occur despite careful and appropriate use. We report the case of a 9-year-old girl with a presenting diagnosis of aplastic anemia following treatment with ethosuximide for absence seizures. Aplastic anemia can now be cured with stem cell transplantation or immunosuppressive therapy. In this case, however, because of the impossibility of bone marrow transplantation and the specific needs of the patient's parents, three courses of methylprednisolone pulse therapy were administered. Following the therapy, there was improvement in pancytopenia and complete remission in the bone marrow. No adverse side effects of therapy were observed. The authors suggest that methylprednisolone pulse therapy may be a treatment for acquired aplastic anemia.


Subject(s)
Anemia, Aplastic , Anticonvulsants , Bone Marrow , Bone Marrow Transplantation , Child , Diagnosis , Environmental Exposure , Epilepsy, Absence , Ethosuximide , Female , Humans , Methylprednisolone , Pancytopenia , Parents , Stem Cell Transplantation
15.
Article in English | WPRIM | ID: wpr-691381

ABSTRACT

<p><b>OBJECTIVE</b>To investigate the potential efficacy of panaxadiol saponins component (PDS-C), a biologically active fraction isolated from total ginsenosides, to reverse chemotherapy-induced myelosuppression and pancytopenia caused by cyclophamide (CTX).</p><p><b>METHODS</b>Mice with myelosuppression induced by CTX were treated with PDS-C at a low- (20 mg/kg), moderate- (40 mg/kg), or high-dose (80 mg/kg) for 7 consecutive days. The level of peripheral white blood cell (WBC), neutrophil (NEU) and platelet (PLT) were measured, the histopathology and colony formation were observed, the protein kinase and transcription factors in hematopoietic cells were determined by immunohistochemical staining and Western blot.</p><p><b>RESULTS</b>In response to PDS-C therapy, the peripheral WBC, NEU and PLT counts of CTX-induced myelosuppressed mice were significantly increased in a dose-dependent manner. Similarly, bone marrow histopathology examination showed reversal of CTX-induced myelosuppression with increase in overall bone marrow cellularity and the number of hematopoietic cells (P<0.01). PDS-C also promoted proliferation of granulocytic and megakaryocyte progenitor cells in CTX-treated mice, as evidenced by significantly increase in colony formation units-granulocytes/monocytes and -megakaryocytes (P<0.01). The enhancement of hematopoiesis by PDS-C appears to be mediated by an intracellular signaling pathway, this was evidenced by the up-regulation of phosphorylated mitogen-activated protein kinase (p-MEK) and extracellular signal-regulated kinases (p-ERK), and receptor tyrosine kinase (C-kit) and globin transcription factor 1 (GATA-1) in hematopoietic cells of CTX-treated mice (P<0.05).</p><p><b>CONCLUSIONS</b>PDS-C possesses hematopoietic growth factor-like activities that promote proliferation and also possibly differentiation of hematopoietic progenitor cells in myelosuppressed mice, probably mediated by a mechanism involving MEK and ERK protein kinases, and C-kit and GATA-1 transcription factors. PDS-C may potentially be a novel treatment of myelosuppression and pancytopenia caused by chemotherapy.</p>


Subject(s)
Animals , Antineoplastic Agents , Cell Proliferation , Cyclophosphamide , Extracellular Signal-Regulated MAP Kinases , Metabolism , GATA1 Transcription Factor , Metabolism , Ginsenosides , Pharmacology , Therapeutic Uses , Hematopoiesis , Mice , Mitogen-Activated Protein Kinase Kinases , Metabolism , Myeloid Cells , Pathology , Panax , Chemistry , Pancytopenia , Drug Therapy , Pathology , Phosphorylation , Proto-Oncogene Proteins c-kit , Metabolism , Saponins , Pharmacology , Up-Regulation
16.
Article in English | WPRIM | ID: wpr-760476

ABSTRACT

In patients with acute myeloid leukemia (AML), pleural effusion may be attributed to various factors, including infection, hypoalbuminemia, and renal failure. However, leukemic infiltration of the pleural fluid is rarely reported and poorly understood. Extramedullary diseases have been reported with increasing frequency as the survival rates of patients with AML have increased. However, the reported prognostic effects of leukemic pleural effusion in patients with AML range from none to a worse prognosis. Here, we report a case of acute promyelocytic leukemia (APL) in a patient exhibiting leukemic pleural effusion with fluorescence in situ hybridization (FISH) results indicating the presence of the PML-RARA fusion gene. A 52-year-old man presented with pancytopenia, dyspnea, and fever. He had a medical history of hypertension, end-stage renal disease, and hepatitis B virus-related liver cirrhosis. A peripheral blood smear revealed the presence of multiple abnormally hypergranular promyelocytes. White blood cell differential counts were not performed due to severe pancytopenia. A bone marrow examination, immunophenotyping analysis, and cytogenetic and molecular studies revealed APL. The patient was treated with all-trans retinoic acid immediately after abnormal promyelocytes were observed in the peripheral blood smear, but induction chemotherapy was delayed because of his poor condition. His persistent dyspnea and abdominal discomfort led to a thoracentesis and the observation of abnormal promyelocytes that were positive for PML-RARA fusion gene by FISH. To our knowledge, this is the first report of leukemic pleural infiltration with PML-RARA fusion gene-positivity via FISH.


Subject(s)
Bone Marrow Examination , Cytogenetics , Dyspnea , Fever , Fluorescence , Granulocyte Precursor Cells , Hepatitis B , Humans , Hypertension , Hypoalbuminemia , Immunophenotyping , In Situ Hybridization , Induction Chemotherapy , Kidney Failure, Chronic , Leukemia, Myeloid, Acute , Leukemia, Promyelocytic, Acute , Leukemic Infiltration , Leukocytes , Liver Cirrhosis , Middle Aged , Pancytopenia , Pleural Effusion , Prognosis , Renal Insufficiency , Survival Rate , Thoracentesis , Tretinoin
17.
Article in English | WPRIM | ID: wpr-762489

ABSTRACT

BACKGROUND: In Korea, there were repeated radiation exposure accidents among non-destructive testing workers. Most of the cases involved local injury, such as radiation burns or hematopoietic cancer. Herein, we report a case of acute radiation syndrome caused by short periods of high exposure to ionizing radiation. CASE PRESENTATION: In January 2017, Korea Information System on Occupational Exposure (KISOE) found that a 31-year-old man who had worked in a non-destructive testing company had been overexposed to radiation. The patient complained of symptoms of anorexia, general weakness, prostration, and mild dizziness for several days. He was anemic. The venous injection areas had bruises and bleeding tendency. Blood and bone marrow testing showed pancytopenia and the patient was diagnosed with acute radiation syndrome (white blood cells: 1400/cubic mm, hemoglobin: 7.1 g/dL, platelets: 14000/cubic mm). He was immediately prohibited from working and blood transfusion was commenced. The patient’s radiation exposure dose was over 1.4 Gy (95% confidence limits: 1.1–1.6) in lymphocyte depletion kinetics. It was revealed that the patient had been performing non-destructive tests without radiation shielding when working in high places of the large pipe surface. CONCLUSIONS: Exposure prevention is clearly possible in radiation-exposed workers. Strict legal amendments to safety procedures are essential to prevent repeated radiation exposure accidents.


Subject(s)
Acute Radiation Syndrome , Adult , Anorexia , Blood Cells , Blood Transfusion , Bone Marrow , Burns , Contusions , Dizziness , Hemorrhage , Humans , Information Systems , Kinetics , Korea , Lymphocyte Depletion , Occupational Exposure , Pancytopenia , Radiation Exposure , Radiation, Ionizing
18.
Article in Korean | WPRIM | ID: wpr-766487

ABSTRACT

Is it necessary to intake anthelmintics every year in Korea? To answer to this question, the recent nation-wide egg positive rate of the intestinal nematodes in Korea was presented. The anthelminthics which are purchasable without physician's prescription were also introduced with their pharmacological reaction and indication. The egg positive rate of Ascaris lumbricoides in 2012 was 0.025%. Those of Trichuris trichiura and Enterobius vermicularis were 0.27% and 0.004%, respectively. In 2018, purchasable anthelmintics without physician's prescription in Korea were albendazole and flubendazole only. Those two anthelmintics were derivatives of benzimidazole that may cause some side effects such as hepatitis, increase of hepatic enzymes, granulocytopenia, or pancytopenia. These anthelmintics showed excellent effect to ascariasis; while, they are not sufficient to treat trichuriaiss. For treatment of enterobiasis, repeated taking 3 times with 3 weeks interval and mass treatment of the family of egg positive person are required. In conclusion, it is not necessary to take anthelmintics every year without specific diagnosis because of negligible egg positive rate of intestinal nematodes and complicated therapeutic module for enterobiasis. There was no specific symptom of ascariasis or trchuriasis if worm burden is not high. The common symptoms of enterobiasis were pain or itching at the perianal area, sleep difficulty, or diarrhea. If intestinal nematode infection is suspected, stool examination or perianal swab should be done before prescribing anthelmintics.


Subject(s)
Agranulocytosis , Albendazole , Anthelmintics , Ascariasis , Ascaris lumbricoides , Diagnosis , Diarrhea , Enterobiasis , Enterobius , Hepatitis , Humans , Korea , Nematode Infections , Ovum , Pancytopenia , Prescriptions , Pruritus , Trichuris
19.
Article in Korean | WPRIM | ID: wpr-787303

ABSTRACT

Osteopetrosis is characterized by impaired osteoclast function and increased bone density. Infantile osteopetrosis is a severe form of the disease and has characteristics such as diffusely sclerotic skeleton, pancytopenia, cranial nerve entrapment, infection susceptibility, and abnormal craniofacial appearance. Patients with infantile osteopetrosis often experience developmental delay, and may have a short life span.A 14-month-old girl with osteopetrosis presented to the department of pediatric dentistry. Incipient caries on deciduous incisors were observed. The patient revisited 4 years of age. Besides medical problems, oral complications such as growth retardation, narrow upper arch, crowding, dental caries, and abnormal tooth development were observed. After consultation with her pediatrician, dental treatments were performed on the deciduous molars under sedation after a prophylactic antibiotic injection. At a periodic follow-up, multiple deciduous teeth were treated and extracted, and oral-rehabilitation with a removable partial denture was initiated.Patient with osteopetrosis are highly susceptible to infection because of their compromised immune system and problems associated with wound healing that lead to osteomyelitis or sepsis development.Active participation in dental care for sugar intake management and proper oral hygiene are obligatory.


Subject(s)
Bone Density , Cranial Nerves , Crowding , Dental Care , Dental Caries , Denture, Partial, Removable , Female , Follow-Up Studies , Humans , Immune System , Incisor , Infant , Molar , Oral Hygiene , Osteoclasts , Osteomyelitis , Osteopetrosis , Pancytopenia , Pediatric Dentistry , Sepsis , Skeleton , Tooth , Tooth, Deciduous , Wound Healing
20.
Article in English | WPRIM | ID: wpr-713248

ABSTRACT

Spontaneous chronic subdural hematoma (SDH) is a rare condition that could develop in association with hematologic disease. A 66-year-old male developed a chronic SDH as an initial manifestation of chronic myelomonocytic leukemia (CMML). He experienced recurrent chronic subdural hemorrhage and newly developed intracerebral hemorrhage. Considering the scheduled long-term chemotherapy, bilateral middle meningeal artery (MMA) embolization was performed to prevent recurrence of subdural hemorrhage. Although pancytopenia occurred during the 7 months' follow-up period, residual chronic subdural hemorrhage was absorbed without recurrence. To our best knowledge, this is the first report of CMML with spontaneous chronic SDH. MMA embolization is potentially a useful and safe treatment option in the challenging clinical situations with underlying pathologies.


Subject(s)
Aged , Cerebral Hemorrhage , Drug Therapy , Follow-Up Studies , Hematologic Diseases , Hematoma, Subdural , Hematoma, Subdural, Chronic , Humans , Leukemia , Leukemia, Myelomonocytic, Chronic , Male , Meningeal Arteries , Pancytopenia , Pathology , Recurrence
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