Manifestações orofaciais associadas aos diferentes tipos de anemias / Orofacial manifestations associated with different types of anemia

Introduction: Anemias correspond to hematological disorders that can present in the oral cavity and face. Objective: To review the literature on the main types of anemic disorders and their orofacial manifestations, considering the aspects of interest to dentists. Methodology: This is a literature review, in which articles were selected in Portuguese and English, indexed in the Scielo, Medline/Pubmed and Lilacs databases with the descriptors: Anemia, Oral Manifestations, Jaw Abnormalities and their correspondents in Portuguese language. Literature review: Anemic disorders associated with orofacial signs and symptoms include mainly Iron-Deficiency, Megaloblastic, Fanconis, Sickle Cell, Thalassemia and Aplastic Anemia. The manifestations vary from burning and painful symptoms in the tongue, pallor of lips and mucosa, stomatitis, atrophic glossitis, angular cheilitis, susceptibility to candidiasis and peri-odontal disease. Also, dental changes, hyposalivation, malocclusion, osteomyelitis of the jaw, paraesthesia of the mental nerve and orofacial pain are included. Conclusion: These manifestations can be the first signs of the presence of anemia, which gives the dentist an important role in early diagnosis and proper management of dental treatment.

Introdução: As anemias correspondem a distúrbios hematológicos que podem apresentar manifestações na cavidade oral e face. Objetivo: Revisar a literatura acerca dos principais tipos de distúrbios anêmicos e suas manifestações orofaciais, considerando os aspectos de interesse aos cirurgiões-dentistas. Metodologia: Trata-se de uma revisão de literatura, em que foram selecionados artigos em português e inglês, indexados nas bases de dados do Scielo, Medline/Pubmed e no Lilacs, com os descritores: Anemia, Oral Manifestations, Jaw Abnormalities e seus correspondentes na língua portuguesa. Revisão de literatura: Os distúrbios anêmicos associados aos sinais e sintomas orofaciais incluem principalmente a Anemia Ferropriva, Megaloblástica, de Fanconi, Falciforme, Talassemia e Anemia Aplástica. As manifestações variam de ardência e sintomatologia dolorosa em língua, palidez de lábios e mucosa, estomatite, glossite atrófica, queilite angular, suscetibilidade a candidíase e doença periodontal. Ainda, englobam-se as alterações dentárias, hipossalivação, má oclusão, osteomielite da mandíbula, parestesia do nervo mental e dor orofacial. Conclusão: Essas alterações podem ser os primeiros sinais da presença da anemia, o que confere ao cirurgião-dentista um importante papel no seu diagnóstico precoce e condução adequada ao tratamento odontológico.

Aplasies médullaires de l'enfant au CHU de Brazzaville

Introduction : L'Aplasie Médullaire (AM) est une insuffisance quantitative de l'hématopoïèse responsable d'une pancytopénie avec une moelle osseuse pauvre. Elle peut être constitutionnelle ou acquise (toxique, infectieuse, idiopathique). Si le traitement est bien codifié dans les pays riches avec l'amélioration du pronostic à long terme depuis plusieurs années, cette maladie demeure hautement mortelle dans les pays en voie de développement.Objectif : Etudier les caractéristiques cliniques, thérapeutiques et évolutives de l'AM chez l'enfant au CHU de Brazzaville.Matériels et méthodes : Il s'est agi d'une cohorte historique, réalisée sur la base des dossiers d'enfants hospitalisés pour une AM sur une période de 15 ans (2001- 2015). La classification de CAMITTA avait été utilisée.Résultats : 22 dossiers avaient été retenus. La moyenne d'âge était de 12,16 ans. Le sex-ratio garçon/fille était de 0,8. Les AM étaient toutes idiopathiques. Le syndrome anémique était le principal motif d'admission. Le taux moyen d'hémoglobine était de 4,3 g/dL, celui des polynucléaires neutrophiles de 0,340 Giga/l et celui des plaquettes de 13,2 Giga/l. Seize patients sur 22 (72,72%) souffraient de la forme sévère. Dans 90,90% des cas le traitement était symptomatique (concentrés érythrocytaires et plaquettaires, antibiothérapie) parfois associé à la corticothérapie ; 2 patients/22 (9,10%) ont reçu de la ciclosporine. Le taux de mortalité était de 94,73% après un suivi moyen de 9 semaines.Conclusion : L'AM de l'enfant au CHU de Brazzaville est essentiellement idiopathique et de pronostic redoutable

Clinical Relevance of p53 Immunohistochemical Stain in the Differential Diagnosis Between Pediatric Aplastic Anemia and Refractory Cytopenia of Childhood

No abstract available.

Anemia aplásica asociada a hepatitis E: Informe de un caso

La aplasia medular asociada a hepatitis (HAAA) es una reconocida entidad clínica donde la falla medular es precedida de una hepatitis; se observa hasta en el 5% de las aplasias en Europa occidental y América del Norte y hasta en el 10% de ellas en el Este asiático. Se ha sospechado de los virus hepatotropos y otros virus como responsables de HAAA, pero esta asociación raramente se ha confirmado. La hepatitis por virus E es la causa más frecuente de hepatitis viral en el mundo. Su genotipo 3, de mayor circulación en la Argentina y otros países de Latinoamérica, puede presentar complicaciones extrahepáticas (renales, neurológicas, pancreáticas y hematológicas). Hasta aquí, en nuestro conocimiento solo se ha publicado un caso de HAAA por virus de la hepatitis E en Pakistán; el que ahora presentamos sería el primero comunicado en la Argentina. La paciente fue tratada con timoglobulina, ciclosporina, corticosteroides, filgastrim y soporte transfusional. Desarrolló fungemia por Candida tropicalis que respondió a equinocandinas, y luego infiltrados pulmonares e imagen nodular cerebral con galactomananos en suero (índice DO > 1.0 ng/ml) que resolvieron con voriconazol. Fue dada de alta independiente de transfusiones, tres meses después de su admisión, con hepatograma normal. Teniendo en cuenta este caso, sería conveniente investigar la hepatitis E como causa de HAAA en la Argentina.

Hepatitis-associated aplastic anemia (HAAA) is a well-recognized clinical syndrome in which marrow failure follows the development of hepatitis; it can be observed in up to 5% in the aplastic anemia in West Europe and North American countries and 10% in the East Asia. Although hepatotropic and other viruses were suspected of causing HAAA, this hypothesis was rarely confirmed. Currently, the infection with hepatitis E virus represents the first cause of acute hepatitis in the world. Its genotype 3, the most frequent in Argentina and other Latin American countries, was associated with extrahepatic complications (renal, pancreatic, neurologic and hematologic). To our knowledge, only one case of hepatitis E virus-associated aplastic anemia has been previously reported, in Pakistan; the case presented here would be the first in Argentina. The patient was treated with thymoglobulin, cyclosporine, corticosteroids, filgastrim and transfusional support. She developed fungemia due to Candida tropicalis that remitted with equinocandins and therefore fever, pulmonary infiltrates and a solitary nodular cerebral image with serum galactomannan (DO index > 1.0 ng/ml) that resolved with voriconazol. She was discharged three months after her admission without transfusion requirements and normal hepatic values.With this in mind, it would be advisable to investigate hepatitis E (HEV) as a cause of HAAA in Argentina.

CD34 and p53 Immunohistochemical Stains Differentiate Hypocellular Myelodysplastic Syndrome (hMDS) from Aplastic Anemia and a CD34 Immunohistochemical Stain Provides Useful Survival Information for hMDS

BACKGROUND: The presence of significant dysplasia in bone marrow (BM) aspirates helps to distinguish between hypocellular myelodysplastic syndrome (hMDS) and aplastic anemia (AA). Occasionally, diluted BM aspirates make it difficult to recognize dysplastic changes and can also negatively affect the detection of cytogenetic abnormalities in hMDS. We evaluated the usefulness of CD34 and p53 immunoreactivity for discriminating between hMDS and AA and for estimating survival outcomes in hMDS patients. METHODS: BM clot section (BMC) or BM biopsy (BMB) specimens were obtained from 64 hMDS/AA patients (33 with hMDS and 31 with AA) and seven controls. Immunohistochemical (IHC) staining for CD34 and p53 was performed by using the EnVision detection system (Dako, Denmark). We compared the results of IHC staining, BM findings, and chromosomal analyses, and determined overall survival outcomes. RESULTS: The number of CD34- and p53-positive BM cells was higher among the patients with hMDS than among the patients with AA (P<0.001 and P=0.001, respectively). hMDS patients with increased CD34-positive cells had significantly poorer survival outcomes compared with those with normal number of CD34-positive cells (P=0.013). CONCLUSIONS: CD34 and p53 IHC stains of BMC or BMB provide useful information for differentiating between hMDS and AA. CD34 IHC staining of BMC or BMB also provides useful information for estimating survival outcomes in hMDS patients.

Evaluation of concentration and storage effects of mitomycin C in the diagnosis of Fanconi anemia among idiopatic aplastic anemia patients.

BACKGROUND: Fanconi anemia (FA) is a rare autosomal recessive genetic disorder that shows an increased sensitivity to the intercalating agents such as mytomycin C (MMC), measured as chromosomal aberrations. This study was conducted to differentiate between FA and “idiopathic” aplastic anemia on the basis of induced chromosomal breakage study with MMC. MATERIALS AND METHODS: MMC stress tests in different final concentrations of 20 and 50 ng/ml of MMC were conducted on peripheral blood lymphocytes from 32 patients with aplastic anemia and 13 healthy controls. Fifty nanograms per milliliter of MMC from old, fresh and frozen stocks was used to check the sensitivity of diagnosis on FA-diagnosed patients. Statistical analysis was used for the assessment of aberrations, including chromatid and chromosome breaks and exchanges. RESULTS: Eight patients (25%) with a very high percentage of chromosomal breakage were diagnosed as FA on the basis of the chromosomal breakage study. Six of these patients exhibited congenital anomalies at presentation, while another two lacked such anomalies or had minor physical problems. Freshly made MMC has shown more sensitivity to detect FA patients compared with frozen or 1-week-old MMC stock. CONCLUSIONS: The study indicates that freshly made MMC stress test provides an unequivocal means of differentiation between FA and “idiopathic” aplastic anemia. Further, the study, the first of its kind from Iran, stresses on the need for conducting this test in all aplastic anemia cases, even those without congenital anomalies, for accurate and timely diagnosis of FA to implement appropriate therapy.

Significance of platelet indices in the diagnosis of immune thrombocytopenic purpura and aplastic anaemia

To compare the platelet indices in thrombocytopenia due to immune thrombocytopenic purpura and aplastic anaemia. This is observational study that was carried out from May 2006 to May 2007 at The Department of Haematology, Armed Forces Institute of Pathology [AFIP] and Armed Forces Bone Marrow Transplant Centre [AFBMTC], Rawalpindi. Seventy six [76] patients of thrombocytopenia were studied, of which 35 were patients of Immune thrombocytopenic purpura [ITP] and 41 patients of Aplastic anaemia [AA]. Platelet indices were measured on peripheral blood using automated haematology analyzer. Based on the bone marrow diagnosis, 35 patients of immune thrombocytopenic purpura and 41 patients of aplastic anaemia were studied. All indices were significantly higher in immune thrombocytopenic purpura than apiasiic anaemia. The mean MPV [Mean Platelet Volume] was 10.5fl in immune thrombocytopenic purpura and 8.6fl in aplastic anaemia [p <0.001], mean PDW [Platelet deviation width] was 15.6fl in immune thrombocytopenic purpura and 10.7fl in aplastic anaemia [p <0.001] and mean P-LCR [Platelet large cell ratio] was 31.2% in immune thrombocytopenic purpura and 17.7% in aplastic anaemia [p <0.001]. Our results suggest that these indices can provide reliable information to clinicians about the underlying etiology of thrombocytopenia and may help to avoid bone marrow examination, which is an invasive procedure, in some patients

A Comparison of Fasting Glucose and HbA1c for the Diagnosis of Diabetes Mellitus Among Korean Adults / 예방의학회지

OBJECTIVES: The American Diabetes Association (ADA) has recently recommended the HbA1c assay as one of four options for making the diagnosis of diabetes mellitus, with a cut-point of > or =6.5%. We compared the HbA1c assay and the fasting plasma glucose level for making the diagnosis of diabetes among Korean adults. METHODS: We analyzed 8710 adults (age 45-74 years), who were not diagnosed as having diabetes mellitus, from the Namwon study population. A fasting plasma glucose level of > or =126 mg/dL and an A1c of > or =6.5% were used for the diagnosis of diabetes. The kappa index of agreement was calculated to measure the agreement between the diagnosis based on the fasting plasma glucose level and the HbA1c. RESULTS: The kappa index of agreement between the fasting plasma glucose level and HbA1c was 0.50. CONCLUSIONS: The agreement between the fasting plasma glucose and HbA1c for the diagnosis of diabetes was moderate for Korean adults.

Successful Salvage Unrelated Umbilical Cord Blood Transplantation with Two Units After Engraftment Failure with Single Unit in Severe Aplastic Anemia

Severe aplastic anemia (SAA) patients without an HLA-matched sibling donor need alternative treatment options. Umbilical cord blood transplantation (UCBT) has become an alternative means for treating various diseases, but it has not been proved to be a satisfactory method to treat SAA. Here, we report the case of a girl who underwent successful two-unit UCBT after engraftment failure with a single unit. Twounit UCBT is proposed to have better engraftment potential and to offer a better chance of survival, according to some reports. Increased cell dose and graft-versus-graft reaction could contribute to these advantages. With this promising result, two-unit UCBT could be an alternative treatment option for patients with SAA without an HLA-matched donor.

Detection of Putative T cell Clones Using T cell Receptor beta Chain Gene Clonality Assay in Korean Patients with Aplastic Anemia / 대한진단검사의학회지

BACKGROUND: We analyzed T cell receptor beta chain (TCRB) gene to investigate the presence of putative T cell clones and its clinicopathologic implications in Korean patients with aplastic anemia (AA). METHODS: Twenty-nine bone marrow specimens were collected from 20 AA patients, 19 specimens from initial diagnosis and 10 from follow-up. T cell clonality assay was performed using IdentiClone(TM) TCRB Gene Clonality Assay kit (InVivoScirbe Technology, USA) and automatic genetic analyzer. Patients' clinical information and laboratory parameters were also analyzed. RESULTS: Five patients had definitive underlying factors related with aplastic anemia, such as hepatitis B virus (4 cases) and benzene exposure (1 case). Putative T cell clones were detected in bone marrow specimens of 11 (58%) out of 19 patients at diagnosis. The location of putative T cell clones of TCRB gene (diversity region, Dbeta; joining region, Jbeta; variable region, Vbeta) was distributed in Dbeta2+Jbeta2 (6 cases), Dbeta1+Jbeta1 (3 cases), Vbeta+Jbeta1 (2 cases), and Dbeta1+Jbeta2 (2 cases). Interestingly, among seven patients who underwent stem cell transplantation, five patients with no T cell clones detected at diagnosis developed new T cell clones during the follow-up. CONCLUSIONS: Putative pathogenetic T cell clones were detected in most of AA patients in the current study. T cell clonality assay would be useful for investigating the pathophysiology of acquired AA.

Pattern of hematological diseases in hospitalized pediatric patients based on bone marrow examination

The objective of this study was to look into various diagnoses of hematological lesions on bone marrow examination in our pediatric age group of patients. This study was conducted in the Pediatric A Unit and Department of Hematology Postgraduate Medical Institute [PGMI] Lady Reading Hospital, Peshawar from 01st Jan 2007 to 31st Dec 2007. Children admitted with pallor, bleeding, lymphadenopathy or visceromegaly having abnormal smear results were included in this study. The data was statistically analyzed by SPSS version 10. One hundred and ninety-eight cases were included in the study. The age range was from 06 months to 14 years with a mean age of 5.35 years and standard deviation of +/- 3.69. Majority [4 7.5%] of these children were in the age range of 1 to 5 years with male to female ratio of 1.53. The commonest disorder was aplastic anemia present in 40 [20.2%] of the cases followed by idiopathic thrombocytopenic purpura [ITP] [15.7%], megaloblastic anemia [14.6%] and iron deficiency anemia [7.6%]. Acute leukemia was the predominant malignant disorder present in 11.6% of the cases. There were also few cases of histiocytosis and bone marrow secondaries. Visceral leishmaniasis, anemia of chronic disorders, haemolytic anemia, myeloid hyperplasia, hypersplenism, congenital dyserythropoietic anemia, malaria and Gaucher disease were the other non-malignant hematological disorders found in this study. Aplastic anemia, idiopathic thrombocytopenic purpura, megaloblastic anemia and leukemia are the commonest hematological disorders in our set up

Differentiation of Fanconi and aplastic anemia using chromosomal breakage test in Southern Iran

Fanconi anemia [FA] is a chromosomal breakage disorder characterized by familial aplastic anemia [AA], various congenital anomalies, and a characteristic chromosomal response to clastogenic stress. In this study, chromosome breakage test was performed for 38 patients suspected of having FA and age-matched controls. According to the results, ten patients were considered as FA cases and 15 patients with no chromosomal breaks were considered as AA. Differentiation of FA from AA is very important because the primary treatment is different. This test should be done in every primary presentation of AA

Posthepatitis aplastic anaemia presenting only with bilateral vision loss.

Aplastic anaemia is pancytopenia with marrow hypocellularity. Hepatitis-associated aplastic anaemia is a varient or aplastic anaemia that follows an acute attack of seronegative hepatitis. Here a case of hepatitis-associated aplastic anaemia presenting with sudden onset of severe simultaneous bilateral vision loss and without any other usual presenting signs is reported. Partial recovery of blood cell count occurred following immunosuppressive therapy. Posterior hyalodotmy helped rapid resolution of premacular subhyaloid haemorrhage. Although bilateral vision loss may rarely be the initial presentation of aplastic anaemia, no such report is known in hepatitis-associated aplastic anaemia. Posthepatitis vision loss needs careful investigation to exclude an underlying haematological disorder.

Hemorragia macular en anemia aplásica. Reporte de un caso / Macular hemorrhage in plastic anemia: A case report

Se presenta el caso de una paciente mujer de 31 años, con diagnóstico de anemia aplásica, quién refería disminución de agudeza visual central en el ojo derecho. Al examen del segmento anterior no había signos de sangrado. A la fundoscopía se encontraron hemorragias retinales en mancha en distinto cuadrante en cada ojo; hemorragia macular que comprometía la fovea en el ojo derecho y hemorragia preretinal con nivel en el cuadrante temporal inferior en el ojo izquierdo. Su evolución fue desfavorable, a la semana, presentó gran compromiso del sensorio, malestar general y al examen oftalmológico se encontraron hematomas subconjuntivales en ambos ojos como signo evolutivo de la severa plaquetopenia.(Rev Med Hered 2007;18:45-48).

Acute myeloid leukemia after intensive immunosuppressive therapy in aplastic anemia.

A 10-year-old boy was admitted with complaints of fever, pallor, fatigue and skin bleeds of 10 days duration and diagnosed as very severe aplastic anemia. He was given intensive immunosuppressive therapy but showed no response to therapy. He later evolved into acute myeloid leukemia. The occurrence of AML is reviewed and possible pathogenesis is discussed.

Aplastic anemia in an HIV infected child.

Hematologic manifestations of HIV in children are common and include anemia, neutropenia, lymphocytopenia, thrombocytopenia that may occur due to many reasons. However, aplastic anemia due to HIV infection is rare and even more so in children. Though anemia is seen with advanced disease and associated with poor prognosis it is treated with various therapeutic modalities. Our patient with aplastic anemia due to HIV infection responded to antiretroviral therapy.