Infección fúngica invasiva por Saprochaete capitata en un niño con aplasia medular / Invasive fungal infection by Saprochaete capitata in a child with bone marrow aplasia

RESUMEN Saprochaete capitata es una causa rara de infección fúngica invasiva en pacientes inmunocomprometidos con alta mortalidad y resistencia antifúngica. Presentamos el caso de un niño de cinco años con diagnóstico de aplasia medular, sometido a trasplante de progenitores hematopoyéticos (TPH), que cursó con neutropenia febril persistente, dolor abdominal intenso, aparición de lesiones maculopapulares en piel y deterioro de la función renal. Se identificó la presencia de S. capitata, en hemocultivos transcatéter venoso central. Esta infección fúngica invasiva resulta ser rara, pero emergente y potencialmente mortal, en pacientes con neutropenia febril persistente y uso prolongado de dispositivos invasivos intravasculares como catéter venoso central.

ABSTRACT Saprochaete capitata is a rare cause of invasive fungal infection in immunocompromised patients with high mortality and antifungal resistance. We present the case of a 5-year-old boy with bone marrow aplasia, who underwent hematopoietic stem cell transplantation (HSCT) and presented persistent febrile neutropenia, abdominal pain, appearance of maculopapular lesions on the skin, and impaired renal function. The presence of S. capitata was identified by blood culture from a central venous catheter. This invasive fungal infection is rare but emergent and life-threatening, especially in immunocompromised patients with persistent febrile neutropenia and prolonged use of invasive devices such as central venous catheters.

Primary cutaneous aspergillosis and idiopathic bone marrow aplasia

Abstract: We describe the case of a 9-year-old boy with idiopathic bone marrow aplasia and severe neutropenia, who developed skin ulcers under cardiac monitoring electrodes. The diagnosis of primary cutaneous aspergillosis was made after the second biopsy and culture. Imaging investigation did not reveal internal fungal infection. The child was treated, but did not improve and died 3 months after admission. The report highlights and discusses the preventable risk of aspergillus skin infection in immunocompromised patients.

A Case of Severe Chronic Active Epstein-Barr Virus Infection with Aplastic Anemia and Hepatitis / 대한소화기학회지

Epstein-Barr virus (EBV) causes various acute and chronic diseases. Chronic active EBV infection (CAEBV) is characterized by infectious mononucleosis-like symptoms that persist for more than 6 months with high viral loads in peripheral blood and/or an unusual pattern of anti-EBV antibodies. Severe CAEBV is associated with poor prognosis with severe symptoms, an extremely high EBV-related antibody titer, and hematologic complications that often include hemophagocytic lymphohistiocytosis. However, CAEBV which led to the development of aplastic anemia (AA) has not been reported yet. A 73-year-old woman was admitted to our hospital with intermittent fever, general weakness and elevated liver enzymes. In the serologic test, EBV-related antibody titer was elevated, and real-time quantitative-PCR in peripheral blood showed viral loads exceeding 10(4) copies/microg DNA. Liver biopsy showed characteristic histopathological changes of EBV hepatitis and in situ hybridization with EBV-encoded RNA-1 was positive for EBV. Pancytopenia was detected in peripheral blood, and the bone marrow aspiration biopsy showed hypocellularity with replacement by adipocytes. AA progressed and the patient was treated with prednisolone but deceased 8 months after the diagnosis due to multiple organ failure and opportunistic infection. Herein, we report a rare case of severe CAEBV in an adult patient accompanied by AA and persistent hepatitis.

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.

Clinicohaematological spectrum of pancytopenia in a tertiary care hospital

To determine the frequency, clinical presentation and underlying causes of pancytopenia inpatients presenting to a tertiary care hospital in Peshawar. This cross-sectional, observational study was conducted at Department of Pathology, Lady Reading Hospital Peshawar from January to December 2011. Patients of all ages having pancytopenia on blood film examination [TLC < 4000/ul, Hb < 10 gm/dl and Platelets < 150000/ul] were included in the study. Already diagnosed patients of Aplastic Anemia, Acute Leukemia receiving treatment and those not willing for bone marrow examination were excluded from the study. History, General Physical and systemic examination were recorded at presentation. The peripheral blood counts were performed with sysmex - automated hematology analyzer. Bone marrow aspiration and trephine biopsy were performed according to the standard protocol and examined microscopically to find the underlying cause of pancytopenia. Other relevant investigations were also done. During the study period, we received 600 patients for bone marrow examination from various units. Out of these, 160 [26.7%] patients had pancytopenia. Common clinical presentations were Pallor[95%, n=150], followed by generalized weakness [75%, n=120], fever [52%, n=83], bleeding manifestation[37.5%, n=60], gastrointestinal symptoms [32.5%, n=52] and splenomegaly [23.5%, n=38]. The common causes of pancytopenia were aplastic anemia [37.5%, n=60] followed by magaloblastic anemia [13.75%,n=22], Acute Leukemia [13.75%, n=22] and hypersplenism [10%, n=16]. Pancytopenia is a common occurrence. Aplastic Anemia and Magaloblastic Anemia are the commonest causes of Pancytopenia followed by Acute Leukemia. Common clinical presentations were Pallor, fever, weakness, bleeding manifestation and Splenomegaly

Rash y anemia aplásica inducidos por fenitoína: caso clínico / Phenytoin-induced rash and aplastic anemia: case report

Rash is a common side effect associated with antiepileptic drugs. The rate of a phenytoin rash is 5.9 percent and increases to 25 percent in those with another antiepileptic drug rash. Aplastic anemia is an adquired hematopoietic stem-cell disorder characterized by pancytopenia of the peripheral blood and hypocellular bone marrow. The use of phenytoin is associated with a 3.5 fold increased risk of aplastic anemia. We report a case of a 70-year-old woman who developed two severe adverse reactions simultaneously with phenytoin: a maculopapular pruritic rash with involvement of mucous and an aplastic anemia. Both conditions normalized after phenytoin withdrawal.

El rash es un efecto secundario común asociado al uso de fármacos antiepilépticos. La frecuencia de rash con fenitoína se ha estimado en un 5,9 por ciento y asciende a un 25 por ciento en pacientes que han presentado rash con otro fármaco antiepiléptico. La anemia aplásica es una anomalía adquirida de las células madre hematopoyéticas caracterizada por pancitopenia de la sangre periférica y médula ósea hipocelular. Los pacientes tratados con fenitoína presentan un riesgo 3,5 veces mayor de desarrollar anemia aplásica. Presentamos el caso de una mujer de 70 años que desarrolló dos reacciones adversas severas y simultáneas a la fenitoína: un exantema maculopapular pruriginoso con compromiso de mucosas y una anemia aplásica. Ambas condiciones se resolvieron completamente con la suspensión del fármaco.

Psoas Abscess Caused by Non-Typhoid Salmonella in a Patient with Severe Aplastic Anemia

The clinical spectrum of infections caused by non-typhoid Salmonella spp. includes gastroenteritis, enteric fever, bacteremia, and extraintestinal localized complications, especially in immunocompromised hosts. Here we report a patient with severe aplastic anemia developing left iliopsoas abscess caused by non-typhoid Salmonella (NTS), which was successfully treated by prolonged antibiotic treatment and repeated debridement. Our data indicate that aplastic anemia is a risk factor for infection caused by NTS.

Aplastic Anemia with Trisomy 8 and Trisomy 9 in Intestinal Behcet's Disease / 대한소화기학회지

Behcet's disease is a multisystemic inflammatory disease characterized with recurrent oral ulcer, genital ulcer, and multiple organ involvement. Aplastic anemia is one of the rarest complications of Behcet's disease. There were only several reports about Behcet's disease associated myelodysplatic syndrome worldwide. Moreover, aplastic anemia in intestinal Behcet's disease was rarely reported. Here, we present a case of aplastic anemia with trisomy 8 and trisomy 9 in intestinal Behcet's disease and a review of the literatures. To the authors' knowledge, this is the first case ever reported in Korea.

Etiological spectrum of pancytopenia based on bone marrow examination in children

To determine the spectrum of pancytopenia with its frequency, common clinical presentation and etiology on the basis of bone marrow examination in children from 2 months to 15 years. Observational study. Department of Paediatrics, Liaquat University of Medical and Health Sciences [LUMHS], Jamshoro, from October 2005 to March 2007. All patients aged 2 months to 15 years having pancytopenia were included. Patients beyond this age limits, already diagnosed cases of aplastic anemia and leukemia, clinical suspicion of genetic or constitutional pancytopenia, history of blood transfusion in recent past, and those not willing for either admission or bone marrow examination were excluded. History, physical and systemic examination and hematological parameters at presentation were recorded. Hematological profile included hemoglobin, total and differential leucocyte count, platelet count, reticulocyte count, peripheral smear and bone marrow aspiration/biopsy. During the study period, out of the 7000 admissions in paediatric ward, 250 patients had pancytopenia on their peripheral blood smear [3.57%]. Out of those, 230 patients were finally studied. Cause of pancytopenia was identified in 220 cases on the basis of bone marrow and other supportive investigations, while 10 cases remained undiagnosed. Most common was aplastic anemia [23.9%], megaloblastic anemia [13.04%], leukemia [13.05%], enteric fever [10.8%], malaria [8.69%] and sepsis [8.69%]. Common clinical presentations were pallor, fever, petechial hemorrhages, visceromegaly and bleeding from nose and gastrointestinal tract. Pancytopenia is a common occurrence in paediatric patients. Though acute leukemia and bone marrow failure were the usual causes of pancytopenia, infections and megaloblastic anemia are easily treatable and reversible

Acquired hemoglobin H disease in a patient with aplastic anemia evolving into acute myeloid leukemia

CONTEXTO: O prognóstico da anemia aplástica grave melhorou com o advento do transplante de medula óssea e do tratamento imunossupressor com globulina antitimocitária. Em contraste com o sucesso destes protocolos, os estudos com seguimento a longo prazo mostraram a ocorrência de doenças clonais, tais como: hemoglobinúria paroxística noturna, síndrome mielodisplásica e leucemia aguda. RELATO DE CASO: Nós relatamos o primeiro caso descrito no Brasil de um paciente com anemia aplástica que evoluiu para síndrome mielodisplásica e leucemia mielóide aguda associada a presença de hemoglobina H e aumento da hemoglobina fetal.

Citologia vaginal a fresco na gravidez: correlação com a citologia corada pela técnica de Papanicolaou / Fresh wet mount in pregnancy: correlation with Pap smears

OBJETIVO: analisar o conteúdo vaginal utilizando o exame citológico a fresco na primeira consulta pré-natal em mulheres com ou sem queixas genitais e correlacionar os resultados com os encontrados na citologia corada pela técnica de Papanicolaou. A microscopia direta durante a gravidez deve ser valorizada e reconhecida como método propedêutico capaz de diagnosticar, de forma imediata, 90 por cento dos casos de vaginose bacteriana, candidose e tricomonose. MÉTODOS: estudo prospectivo em 216 gestantes, selecionadas em ambulatório de pré-natal no período de 30 de outubro de 2001 a 12 de novembro de 2002. Foram colhidas duas amostras do conteúdo existente no fundo de saco vaginal posterior e depositadas em lâminas de vidro para microscopia. Sobre a primeira e a segunda amostra eram colocadas uma gota de NaCl a 0,9 por cento e uma de KOH a 10 por cento, respectivamente. Adicionalmente, em todas as grávidas determinou-se o pH vaginal e realizaram-se os testes de produção das aminas com odores de pescado. O material era examinado ao microscópio em aumentos de 100 vezes, 400 vezes e excepcionalmente 1000 vezes. Foram realizados esfregaços cervicovaginais para citologia corada pelo método de Papanicolaou. A correlação entre os resultados dos métodos citológicos empregados foi realizada pelo cálculo do coeficiente kappa, que avalia a concordância para variáveis qualitativas. RESULTADOS: o encontro nos esfregaços a fresco de flora bacteriana normal foi de 51,8, representando o aspecto citológico mais observado e sem correspondência com os 3,7 por cento apurados na microscopia corada. No exame citológico direto foram observados 30,9 por cento de vaginose bacteriana e 7,9 por cento de candidose. Todavia, no Papanicolaou não foi encontrada tal equivalência, sendo as porcentagens de 0,7 e 24,3 por cento, respectivamente. A ausência de correlação no diagnóstico de colpite bacteriana inespecífica na microscopia direta (17,5 por cento) e corada (51,3 por cento) talvez deva-se ao subdiagnóstico de vaginose neste último método propedêutico. Os diagnósticos de tricomoníase observados em ambos os métodos citológicos (3,7 e 2,7 por cento) traduzem a baixa prevalência destes parasitas na gestação. O cálculo do índice kappa para avaliação da concordância entre os dois procedimentos citológicos nos diversos achados microbiológicos demonstrou baixa correlação nos diagnósticos da vaginose bacteriana e colpites bacterianas inespecíficas, bem como na identificação da flora...

Oro-nasal fistula of infective origin and alveolar necrosis in a 13 year old boy.

An unusual case of oronasal fistulla of infective origin in a patient with a previous history of aplastic anemia and cancrum oris is discussed here in this report. As the general condition of the patient was not favouring the surgical closure, an obturator was fabricated and inserted to relieve the problems of regurgitation of food and fluids into the nasal cavity.

Monosomy 7 in patients with aplastic anemia and paroxysmal nocturnal hemoglobinuria with evolution into acute myeloid leukemia.

We report two cases of Thai patients with aplastic anemia/paroxysmal nocturnal hemoglobinuria (AA/PNH) who subsequently developed acute myeloid leukemia (AML) at their terminal phase. Monosomy 7 was demonstrated upon karyotypic analysis of bone marrow in both cases at the time leukemia developed The first patient was a 25-year-old man diagnosed with AA at age 14, recovered from AA at age 15, developed PNH at age 21 and turned into AML at age 25. The second patient was a 27-year-old man diagnosed with PNH at age 22, developed severe AA at age 25 and turned into AML at age 27. This latter patient received anti-lymphocyte globulin when he developed severe AA but did not respond well whereas the first patient fully recovered from AA with anabolic hormone treatment. Time to diagnosis of AML in the patient who received immunosuppressive therapy was strikingly shorter than that who received conventional androgen therapy (2 years vs 11 years after AA, respectively). The presence of monosomy 7 in leukemic cells of both patients emphasizes its central role in the development of AML from AA/PNH. However, other factors such as choice of AA/PNH therapy and patients response may modulate the time to emergence of monosomy 7-carrying AML clone and frank leukemia. Further studies into the biologic and genetic mechanisms involved in the development of leukemic clone arising from AA/PNH should be explored.

A Case of Focal Segmental Glomerulosclerosis Associated with Aplastic Anemia

The pathogenic mechanism of focal segmental glomerulosclerosis (FSGS) and aplastic anemia are associated with immunologic events which lead to glomerular cell injury or hematopoietic cell destruction. We present an extremely rare case of FSGS with aplastic anemia in a 30-yr-old woman. The laboratory examination show-ed hemoglobin 7.2 g/dL, white blood count of 4,200/microliter, platelet count 70,900/microliter. Proteinuria (2+, 3.6 g/day) and microscopic hematuria were detected in urinalysis. The diagnosis of FSGS and aplastic anemia were confirmed by renal and bone marrow biopsy. She was treated with immunosuppressive therapy of prednisone 60 mg/day orally for 8 weeks and cyclosporine A 15 mg/kg/day orally. She responded with gradually improving her clinical manifestation and increasing peripheral blood cell counts. Prednisone was maintained at the adequate doses with tapering after 8 weeks and cyclosporine was given to achieve trough serum levels of 100-200 ng/mL. At review ten month after diagnosis and initial therapy, the patient was feeling well and her blood cell counts increased to near normal (Hb 9.5 g/dL, Hct 32%, WBC 8,300/microliter, platelet 123,000/microliter) and renal function maintains stable with normal range proteinuria (0.25 g/day).

Osteonecrosis of the Hip in Patients with Aplastic Anemia

The incidence and clinical and magnetic resonance imaging features of osteonecrosis of the hip were evaluated in patients with aplastic anemia. Two hundred and forty-one patients with aplastic anemia were examined using MR imaging of bone marrow during the five years from 1994 to 1998. Osteonecrosis of the hip was observed on MR imaging in nineteen (15 males and 4 females, mean age 35 yr) of the 241 patients. It was present in both hips in 14 patients, and there were five cases with unilateral occurrence, with a total of 33 involved hips. All except for five hips with associated bone marrow edema revealed increased fatty marrow conversion in the proximal femoral metaphysis. In nine patients, osteonecrosis was detected without any pain. Five patients already had osteonecrosis before any medication was administered. Twelve patients received antilymphocyte globulin, and seven patients received a low dose of steroids before the MR diagnosis of osteonecrosis. Osteonecrosis of the hip frequently develops in patients with aplastic anemia (7.9%), associated with fatty marrow conversion of the proximal femoral metaphysis.

Pancytopenia--a clinico-hematologic analysis of 77 cases.

The present study was conducted in the department of Haematology and Transfusion Medicine, Government Medical College & Hospital, Chandigarh over a period of 32 months. During this period pancytopenia was an indication for bone marrow (aspiration and or trephine) in 77 out of a total of 205 cases (37.6%). The most common cause of pancytopenia as revealed by bone marrow was megaloblastic anaemia (68%) followed by aplastic anaemia (7.70%). This study also revealed few uncommon and rare, but interesting causes of pancytopenia like drug induced agranulocytosis, hemophagocytic syndrome and waldenstroms macroglobinemia.