Investigation of the incidence of vitamin B12 deficiency in leukopenia and neutropenia secondary to infection

Background: Infections and nutritional deficiencies are the most common causes of the leucopenia and neutropenia. In this study, we aimed to find the incidence of vitamin B12 deficiency in patients with leukopenia and neutropenia secondary to infection.Methods: Between September 2018 and December 2018, patients who were referred to Diyarbak?r Children Hospital's Pediatric Hematology and Oncology Clinic for leukopenia and/ or neutropenia were thought to develop secondary to infection retrospectively evaluated.Results: Of the 60 patients that evaluated, 23(38.3%) were female and 37(61.7%) were male. The mean age'SD was 4.75'4.5 years. Severe neutropenia was detected in 5(8.3%), moderate neutropenia in 31(51.7%) and mild neutropenia in 17(28.3%) patients. Neutrophil counts were within normal limits in 7(11.7%) patients. When vitamin B12 levels were examined, 17(28.3%) patients had B12 deficiency.Conclusions: Investigation and treatment of vitamin B12 deficiency in patients with leukopenia and neutropenia may shorten the duration of cytopenia and prevent the development of secondary complications.

Linfoma NK/T extra nodal, tipo nasal, con compromiso cutáneo primario: reporte de un caso / Extranodal Natural Killer/T-Cell Lymphoma, nasal-type with primary cutaneous involvement: a case report

INTRODUCCIÓN: El linfoma extranodal natural killer/célula T (NK/T) de tipo nasal, es una neoplasia poco frecuente, con una alta letalidad, caracterizada por destrucción ósea alrededor de los senos paranasales, el septum nasal u obstrucción de la vía aérea. Puede presentar compromiso primario de la piel, vía aérea y otros órganos. OBJETIVO: Presentar un caso ilustrativo de una afección poco frecuente y de curso agresivo en población pediátrica, para facilitar la sospecha diagnóstica y el rápido reconocimiento por parte de los especialistas. CASO CLÍNICO: Adolescente de 14 años, que consultó por lesiones solevantadas en brazos y piernas, no dolorosas, sugerentes de paniculitis subcutánea, las cuales evolucionaron a máculas violáceas ulceradas. La biopsia de las lesiones fue compatible con linfoma NK/T de tipo nasal. Fue derivada a oncología pediátrica, donde recibió tratamiento quimioterápico. Pese a los esfuerzos médicos, la paciente falleció a los 8 meses producto de una infección pulmonar grave secundaria a inmunosupresión. CONCLUSIONES: El linfoma extranodal NK/T, tipo nasal es una neoplasia poco frecuente, que se comporta de forma agresiva, con una alta mortalidad sin tratamiento. Por lo que su reconocimiento es de gran relevancia para el diagnóstico precoz y rápida derivación a Hemato-Oncología.

INTRODUCTION: Extranodal natural killer/T-cell lymphoma (NK/T), nasal type, is an infrequent neoplasm with a high lethality, characterized by bone destruction around the sinus, nasal septum or obstruction of the airway. Also, may be primary skin involvement, airway and other organs. OBJECTIVE: Submit a rare condition in the pediatric population, in order to facilitate the diagnostic suspicion and quick recognition from specialists. CASE REPORT: a 14-year-old girl, who presented arm and leg lesions, painless, suggestive of subcutaneous panniculitis, which evolve to ulcerated purple maculae. Skin biopsy showed lesion compatible with NK/T lymphoma, nasal type. She was referred to pediatric oncology, where she received chemotherapy treatment. Despite medical efforts, the patient died eight months after due to a serious pulmonary infection secondary to immunosuppression. CONCLUSIONS: Extranodal NK/T-cell lymphoma, nasal type, is a rare neoplasm that behaves aggressively, with high mortality without treatment, therefore, its recognition has a high importance for early diagnosis and prompt referral to Hematology-Oncology.

Linfoma tipo hidroa vacciniforme en Pediatría / Hidroa vacciniforme type lymphoma in Pediatrics

Se presenta caso de mujer de 14 años que consultó por úlceras orales y lesiones cutáneas de tipo vesicular, papular y algunas con evolución a úlcerocostras de centro necrótico, hemorrágico y de variadas formas y tamaños, distribuidas principalmente en áreas fotoexpuestas como rostro y extremidades superiores e inferiores. La anatomía patológica confirmó linfoma tipo hidroa vacciniforme atípico, considerado primer caso descrito y publicado en Paraguay.

A 14 year old female consulted with oral ulcers and skin lesions like vesicles and papules, some of them are ulcers and crusting with a necrotic and hemorrhagic center, the shape and size of every lesion is different, mainly they are presented in sun-exposed areas like the face, arms and legs. The histopathological finding confirms the atypical hidro vaccinifome-like lymphoma, considered first report and published in Paraguay.

Epstein-Barr Virus Infection with Acute Acalculous Cholecystitis / 대한소아소화기영양학회지

Acute acalculous cholecystitis (AAC) is an inflammation of the gallbladder in the absence of demonstrated stones. AAC is frequently associated with severe systemic inflammation. However, the exact etiology and pathogenesis of AAC still remain unclear. Acute infection with Epstein Barr virus (EBV) in childhood is usually aymptomatic, whereas it often presents as typical infectious mononucleosis symptoms such as fever, cervical lymphadenopathy, and hepatosplenomegaly. AAC may occur during the course of acute EBV infection, which is rarely encountered in the pediatric population. AAC complicating the course of a primary EBV infection is usually associated with a favorable outcome. Most of the patients recover without any surgical treatment. Therefore, the detection of EBV in AAC would be important for prediction of better prognosis. We describe the case of a 10-year-old child who presented with AAC during the course of primary EBV infection, the first in Korea, and review the relevant literature.

Juvenile Rheumatoid Arthritis in Children with Ebstein-barr Virus Infection

OBJECTIVE: Juvenile rheumatoid arthritis (JRA) may occur in the wake of infection with several viruses including Ebstein-barr virus (EBV). EBV remains an interesting target. To determine the possible role of EBV infections in the clinical course of JRA, we attempt to demonstrate the radiologic changes and the frequency prescription of etanercept rather than classic therapy. METHODS: Total of 87 patients with JRA, who were hospitalized in Hangang Sacred Hospital and Kangnam Sacred Hospital in Seoul from 2002 to 2010, were assessed serologically for EBV infection (anti EBV VCA IgM and IgG) at admission. Patients with JRA were devided 2 groups, one is EBV VCA IgG (+) JRA patients who had been infected before and another is EBV VCA IgG (-) JRA patients who had not. RESULTS: EBV VCA IgG (+) were seen in 55 patients (63.2%). 31 boys (76%) and 24 girls (52%) were infected with EBV. The mean age of patients of EBV (+) JRA was 8.2+/-3.6 years and that of EBV (-) JRA was 5.3+/-3.4 years. 7 of EBV (+) JRA (13%) developed radiologic change within 2 years, compare with none of EBV (-) JRA. 22 of EBV (+) JRA (49%) with JRA did not respond to the classic therapy, compare with 7 of EBV (-) JRA (22%). CONCLUSION: JRA patients with past EBV infection were older in ages, more in male, more radiologic changes, needed more biologic treatment than those without past EBV infection.

Oral Hairy Leukoplakia Which Occurred as a Presenting Sign of Acute Myeloid Leukemia in a Child

Oral hairy leukoplakia (OHL) is caused by the reactivation of a previous Epstein-Barr virus (EBV) infection in the epithelium of the tongue. Most lesions are characterized by corrugated whitish patches on the lateral border of the tongue. It is frequently associated with AIDS, but cases in patients with other immunosuppressed states have also been reported. In leukemia patients, OHL is rarely encountered, and appears only after chemotherapy. We report a case of OHL which occurred as a presenting sign of acute myeloid leukemia (AML) in a previously healthy 15-year-old child. A 15-year-old boy presented with a whitish patch on the left lateral border of the tongue. The biopsy specimen revealed papillomatosis, hyperkeratosis, acanthosis and ballooning degeneration in the stratum spinosum. The patient was EBV seropositive, and PCR analysis of EBV DNA in the lesional tissue was positive. After the diagnosis of OHL in dermatologic department, the patient was referred to pediatrics due to the abnormal peripheral blood smear, and was diagnosed with AML.

Fatal Hemophagocytic Syndrome : Three Autopsy Cases / 대한법의학회지

Hemophagocytic Syndrome (HS) is a systemic lymphohistiocytic proliferative disorder associated with infection or malignancies, particularly Epstein-Barr virus (EBV) HS is presented with high fever, skin rash, hepatosplenomegaly, and cytopenia. The characteristic pathologic finding is massive lymphohistiocytic infiltration with hemophagocytosis in various organs including bone marrow, liver, and spleen. Hyperproduction of cytokines by activated T lymphocytes has been presumed to account for the hemophagocytosis and clinical manifestations of HS. We report three childhood autopsy cases with HS which was confirmed by histopathologic examination. According to medical records, all cases had high fever with or without skin rash, cytopenia, AST/ALT elevation, and hyperfibrinogenemia, and showed multiple organ failure eventually. At autopsy, there were no specific gross findings except splenomegaly, but extensive lymphohistiocytic infiltration with hemophagocytosis was seen in various organs including lymph nodes, spleen, liver, and bone marrow. In one of three cases, EBV was identified with in situ hybridization method. As this disease has rapidly progressive clinical course with fatal outcome during childhood, so the possibility of HS should be considered in children presenting with high fever and hepatosplenomegaly.

A Case of Acute Adrenal Insufficiency with Bilateral Adrenal Hemorrhagic Infarction due to Ebstein-Barr Virus Infection in a Patient with Asymptomatic Chronic Adrenal Insufficiency / 대한내분비학회지

Acute adrenal insufficiency may result from adrenal crisis, hemorrhagic destruction, or the rapid withdrawal of steroids from patient with chronic steroid medication, congenital adrenal hyperplasia or those on other drugs. Acute hemorrhagic destruction of both adrenal glands can occur due to infection, trauma, anticoagulant therapy, antiphospholipid syndrome or a coagulation disorder. However, there have been no reports on acute hemorrhagic adrenal insufficiency due to the Ebstein-Barr virus (EBV). Herein, a case of acute adrenal insufficiency, with bilateral adrenal hemorrhagic infarction, is reported in a patient with asymptomatic chronic adrenal insufficiency. A 42-year-old man presented with general weakness, weight loss and hyperpigmentation of several months duration. He suffered from a sore throat, general myalgia and a headache on admission. The laboratory findings were lymphocytosis, positive EBV IgM antibody, low cortisol level and a high level of adrenocorticotropic hormone (ACTH). Adrenocortical autoantibody and PCR for Mycobacterium tuberculosis showed negative findings. The serologic findings for CMV and HIV were negative. Fine needle aspiration of the adrenal gland revealed a hemorrhagic infarction and positive staining for the anti-EBV antibody. Acute adrenal insufficiency was then diagnosed with a bilateral adrenal hemorrhagic infarction due to the EBV infection in the patient, also with asymptomatic chronic adrenal insufficiency. This is the first case of acute adrenal insufficiency with bilateral hemorrhagic infarction, due to an EBV infection

Influence of Epstin-Barr Virus Infection in Immune Thrombocytopenic Purpura in Childhood / 대한소아혈액종양학회지

PURPOSE: Acute immume thrombocytopenic purpura (ITP) is relatively common hematologic disease in children. Most acute ITP is recovered within 6 month spontaneously and the complication is rare. But 10~20% of the ITP patient became a chronic form. Infection with Epstein-Barr virus (EB virus) in developing country usually occurs during infancy and early childhood. Acute ITP associated with EB virus is likely to develop chronic ITP in current literatures. We studied the pattern of laboratory findings in long term follow up of ITP with EB virus infection. METHODS: One hundred and seventy nine patients diagnosed with ITP admitted to the division of pediatric hematology, Gachon Medical Center and Hanyang University Hospital between Mar. 1991 and Jun. 2001 were reviewed retrospectively. Serologic test for EB virus was available for 57 patients and 25 of them were follow up at least 6 months. Evidence of acute EB virus infection was defined as a positive Viral Capsid Antigen (VCA) IgM or positive Anti VCA IgG and negative Ebstein-Barr virus Nuclear Antigen (EBNA). Complete remission (CR) was defined as a recovery of platelet count of more than 100 109/L and partial remission (PR) as a recovery of platelet count of 50~100 109/L, maintained for at least 6 months. RESULTS: Sixteen out of 57 patients were associated acute EB virus infection. Of this group, 8 patients were follow up at least 6 months. Forty one of 57 with no evidence of acute EB virus infection, 17 were follow up at least 6 months. The clinical and laboratory data was not different significantly in children with and without acute EB virus infection in admission. In EB virus infection group of 6 months follow up, platelet count was significantly lower than control group in 6 months follow up (P=0.006). Five patients of 8 (63%) with acute EB virus infection had chronic ITP and 2 of 17 (12%) with no evidence of EB virus infection had chronic ITP in follow up 6 months. CONCLUSION: Patients with EB virus associated ITP tended to resolved more slowly than those without EB virus infection and also showed tendency to become chronic ITP.

Two cases of posttransplantation lymphoproliferative disorders in recipients of liver transplantation / 대한내과학회지

The posttransplantation lymphoproliferative disorders (PTLD) are not rare complications of solid organ transplants. The incidence varies with the type of transplantation and the nature and intensity of the immunosuppressive regimens. PTLDs are unique in that they have a predilection for extranodal sites, a strong and probably causal association with Ebstein-Barr virus infection, and a poor response to the cytolytic chemotherapeutic or irradiation regimens used for treatment of malignant lymphoma. The outcomes of treatment have been disappointing, with mortality from PTLD or related complications of over 50% of patients. We experienced two cases of PTLDs in liver transplant recipients presenting with liver mass and intraabdominal lymphadenopathy. PTLDs were diagnosed by autopsy and a liver biopsy. In the case diagnosed by a liver biopsy, EBV was detected by in situ hybridization. Despite reduction of immunosuppression and conservative management, both patients died.

Erythroleukemia Related to Chronic Ebstein-Barr Virus Infection / 대한소아혈액종양학회지

It is well known that the Ebstein-Barr virus causes not only infectious mononucleosis in the most cases, but also occasionally causes a variety of hematologic disorders such as anemia (hemolytic anemia or aplastic anemia), thrombocytopenia, neutropenia and malignancies such as Burkitt lymphoma and nasopharyngeal carcinoma. Furthermore, recently it is has been reported that it causes leukemia even though very rarely. The authors report a case of erythroleukemia possibly related to chronic Ebstein-Barr virus infection in a 14-year-old boy with brief review of related literatures.