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Biomedical subjects

Elpis Mantadakis

Publications and source records attributed to Elpis Mantadakis.

At least 19 recordsLinked to original sources

T-cell acute lymphoblastic leukemia relapsing as acute myelogenous leukemia.

We present the unusual case of a 16-year-old girl with T-cell acute lymphoblastic leukemia (ALL) with an early thymocyte immunophenotype without myeloid markers, who after 13 months of complete hematological remission relapsed as acute myelogenous leukemia (AML) with minimal differentiation and died of her disease. Whether the AML represented a relapse with lineage switch of the original immature T-cell clone or a new secondary malignancy, could not be proven due to the absence of molecular or clonal markers. This report suggests that a subset of CD7+ T-cell leukemias without mature T-cell antigens (CD4-, CD8-) are minimally differentiated and can relapse as AML.

Acute Disease↗

Fatal Fournier's gangrene in a young adult with acute lymphoblastic leukemia.

Fournier's gangrene (FG) is a fulminant necrotizing fasciitis of the external genitalia. Few reports of FG exist in patients with hematologic malignancies. We describe a case of fatal FG in a 21-year-old man with acute lymphoblastic leukemia who was receiving remission-induction chemotherapy. Despite early local surgery, administration of appropriate antibiotics, resurgery for wider debridement and aggressive ICU support he succumbed while pancytopenic to septic shock, 26 days after initiation of chemotherapy. Multi-drug resistant Pseudomonas aeruginosa was isolated from blood and scrotal cultures obtained at initial surgery. FG is a fulminant infection, especially in the face of profound cytopenias.

Adult↗

Genital tuberculosis in a tamoxifen-treated postmenopausal woman with breast cancer and bloody vaginal discharge.

BACKGROUND: Female genital tuberculosis is an uncommon disease that is rarely diagnosed in developed countries. CASE PRESENTATION: A 61-year-old postmenopausal woman who had undergone surgery and treated with adjuvant chemotherapy for infiltrating ductal carcinoma of the breast five years ago, presented with bloody vaginal discharge, fatigue, weight loss, and low grade fevers at night for two months. Histological examination of the endometrium, done based on the suspicion of a second primary cancer due to the tamoxifen therapy, revealed a granulomatous reaction. Liquid and solid mycobacterial cultures of the tissues were performed. Although the acid fast staining was negative, the liquid culture was positive for Mycobacterium tuberculosis. Involvement of other systems was not detected. The patient was treated with a three-drug antituberculosis regimen for 9 months and recovered fully. CONCLUSION: Female genital tuberculosis is a rare but curable disease that should be included in the differential diagnosis of women with menstrual problems. Early diagnosis is important and may prevent unnecessary invasive procedures for the patient.

Anti-Bacterial Agents↗

Near demise of a child with Prader-Willi syndrome during elective orchidopexy.

The case of a morbidly obese 3.5-year-old boy, with Prader-Willi syndrome (PWS), who experienced a life-threatening episode of pulmonary edema soon after induction of general anesthesia with sevoflurane and intubation for orchidopexy is presented. The patient who had history of sleep apnea and who had an uneventful laparoscopy under general anesthesia 6 months previously was supported with mechanical ventilation with positive end expiratory pressure but developed hyperthermia, pneumonia, sepsis, and Acute Respiratory Distress Syndrome in the intensive care unit. He recovered fully 11 days after surgery. The possible contributing factors for the development of pulmonary edema are discussed. Arrangements for monitoring in an intensive care setting after surgery are highly recommended for patients with PWS.

Anesthesia, General↗

Transient loss of the Y-chromosome in an elderly man with anemia and lead poisoning: chance occurrence or a clonal marker of the underlying hematological abnormality?

One of the most important environmental and occupational pollutants is lead. Cytogenetic damage is known to occur to many individuals exposed to lead, e.g., outdoor and car painters, traffic policemen, gasoline station attendants, etc.1 Chronic lead exposure affects many organ systems leading to a gradual decline in the so-called safe blood lead levels over time.

Aged↗

Primary cutaneous nocardiosis in 2 patients on immunosuppressants.

Two female cases of primary cutaneous nocardiosis due to Nocardia brasiliensis are described. The first was associated with polymyositis and the second with chronic immune thrombocytopenic purpura. Both patients had received corticosteroids. In both cases the responsible actinomycetes were sensitive to trimethoprim/sulfamethoxazole. This drug was administered to both patients with excellent results. Treatment was continued for 3 months to prevent recurrence, a common consequence of short-term therapy. N. brasiliensis should be included in the differential diagnosis of any case of nodular lymphangitis, especially in immunocompromized patients.

Anti-Bacterial Agents↗

Agnogenic myeloid metaplasia with pulmonary hematopoiesis.

Agnogenic myeloid metaplasia (AMM) is characterized by bone marrow fibrosis, splenomegaly and leukoerythroblastic anemia and is frequently accompanied by extramedullary hematopoiesis (EMH). Pulmonary interstitial EMH associated with myelofibrosis has rarely been described in the medical literature and is usually fatal. We report the case of a 77-year-old man with agnogenic myeloid metaplasia (AMM) treated with hydroxyurea, who seven years after diagnosis presented with dyspnea and severe hypoxemia. Radionuclide bone marrow scanning demonstrated increased tracer activity on the bases of both lungs, consistent with non-hepatosplenic EMH. Pulmonary EMH is rare in patients with AMM, but should be considered in patients with hypoxemia and respiratory distress.

Aged↗

Life-threatening hypercalcemia complicated by pancreatitis in a child with acute lymphoblastic leukemia.

The authors describe a 9-year-old girl with precursor-B acute lymphoblastic leukemia (ALL) who presented with dehydration and severe hypercalcemia. She had received oral vitamin D and calcium supplementation for 4 days, the last dose 48 hours prior to admission, and required pediatric intensive care unit (PICU) hospitalization for management of the hypercalcemia and safe initiation of induction chemotherapy. Her clinical course was complicated by pancreatitis, disseminated intravascular coagulation, pleural effusion, and focal seizures. Although the exact mechanism of hypercalcemia was not elucidated, it was likely related to the underlying ALL, without dismissing the prior vitamin D and calcium supplementation as a possible contributing factor. The hypercalcemia resolved with specific antileukemic therapy along with supportive care and administration of calcitonin. Hypercalcemia is an uncommon metabolic abnormality in children with ALL, but it can be life-threatening. Children with ALL should be referred to tertiary-care institutions with PICU and subspecialty support because serious metabolic and other complications can occur before or after the administration of chemotherapy.

Acute Disease↗

Fulminant hepatitis due to varicella zoster virus in a girl with acute lymphoblastic leukemia in remission: report of a case and review.

The authors describe a 4-year-old girl with acute lymphoblastic leukemia in remission who developed fulminant hepatic failure due to varicella-zoster virus (VZV). Diagnosing VZV visceral infection in immunocompromised patients is often difficult due to atypical clinical presentation with few or no skin lesions and severe abdominal or back pain. Prompt initiation of empirical treatment with acyclovir and VZV immunoglobulin pending results of the serum polymerase chain reaction for VZV is warranted in this clinical setting.

Antineoplastic Combined Chemotherapy Protocols↗

Assessment of megakaryopoiesis in children with acute lymphoblastic leukemia.

The effect of the underlying disease and chemotherapy on megakaryopoiesis has not been extensively studied in children with acute lymphoblastic leukemia (ALL) during and at the end of therapy. Using a serum-free assay, we assessed the megakaryocyte (Mk) colony formation in vitro from bone marrow mononuclear cells of 25 children with ALL during chemotherapy and shortly after the cessation of it. Twelve children with solid tumors without bone marrow involvement and cord blood from 10 full-term normal vaginal deliveries were used as controls. A significant reduction in the number of Mk colonies was observed at diagnosis of ALL, and Mk colony formation remained lower than controls throughout the different phases of leukemia treatment. Our study suggests that defects in megakaryopoiesis of children with ALL in long-term remission may persist during chemotherapy and at least shortly after the end of it.

Child↗

High-dose methotrexate in acute lymphoblastic leukemia: where is the evidence for its continued use?

High-dose intravenous methotrexate is an important component of many effective chemotherapeutic regimens for childhood acute lymphoblastic leukemia (ALL). Its use has a strong pharmacologic rationale: to overcome mechanisms of resistance of the malignant cells and to achieve cytotoxic concentrations in sanctuary sites for lymphoblasts. Although therapeutic progress in ALL during the past 4 decades has been closely associated with more widespread use of intravenous methotrexate and in progressively larger doses, little data exist to clearly support the use of high-dose intravenous methotrexate over a regimen of prolonged administration of low-dose methotrexate. The implied superiority of intravenous methotrexate mainly stems from studies that used identical leucovorin rescue with low-dose methotrexate or from studies of upfront window therapy in untreated children with ALL in which single standard doses of oral methotrexate were compared with high-dose intravenous methotrexate with leucovorin rescue. The evidence favoring administration of intravenous methotrexate for children with ALL is critically reviewed. Despite its extensive use, high-dose intravenous methotrexate has not been proved conclusively to be more effective than less toxic, less labor intensive, and less costly methods of methotrexate administration.

Animals↗

Rifampin plus pyrazinamide-induced hepatitis requiring hospitalization in a 30-y-old male with latent tuberculosis.

The case of a 30-y-old male with latent tuberculosis who developed chemical hepatitis requiring hospitalization after 50 d of treatment with rifampin and pyrazinamide is reported. This case justifies the CDC guidelines that were updated on 31 August 2003 advising against the use of this brief regimen for patients with latent tuberculosis due to its risk for hepatotoxicity.

Adult↗

Rituximab for refractory Evans syndrome and other immune-mediated hematologic diseases.

The authors describe a 21-year-old man with long-lasting Evans syndrome refractory to corticosteroids and immunosuppressive agents; the patient responded to four weekly infusions of rituximab. The patient relapsed with thrombocytopenia 7 months post-therapy and was successfully re-treated with two weekly doses of the same monoclonal antibody. He remains in remission for 7-plus months after the second treatment. Therapy was well tolerated, and no infectious complications occurred, despite avoiding administration of prophylactic gammaglobulin. Rituximab appears safe and modestly effective in a variety of immune-mediated hematologic diseases, including autoimmune hemolytic anemia, chronic immune thrombocytopenia, Evans syndrome, pure red cell aplasia, mixed type II cryoglobulinemia, cold agglutinin disease, and Waldenstrom's macroglobulinemia. However, as most of the published literature consists of case reports and small case series, international collaboration is essential in order to better define the efficacy and safety of this agent in children and adults with hematologic diseases.

Adult↗

Fatal Nocardia farcinica bacteremia in a patient with lung cancer.

Nocardia farcinica is an emerging pathogen in immunocompromised patients, accounting for approximately 20% of Nocardia clinical isolates in various countries. A case of fatal N. farcinica bacteremia in a 52-year-old man with lung cancer is described. He was admitted with severe respiratory distress, and despite the early onset of empirical antibiotic treatment, he failed to respond and died of septic shock 24 hours later. N. farcinica was isolated from blood cultures obtained at hospital admission and was identified by conventional methods. Strain identification was confirmed by nucleotide sequencing of the 16S rRNA gene. N. farcinica bacteremia is a life-threatening infection. Because of the actinomycete's highly-resistant antibiotic profile, early identification and antibiotic susceptibility testing are necessary to improve the chances of survival.

Anti-Bacterial Agents↗

Impact of prolonged treatment with trimethoprim-sulfamethoxazole on the human gut flora.

The case of a mentally ill man inadvertently treated with trimethoprim-sulfamethoxazole (TMP-SMX) for 2 y is presented. Quantitative stool cultures revealed a substantially suppressed Gram-negative aerobic flora, while Enterococcus spp. and anaerobes were not affected. Yeasts were moderately increased. TMP-SMX represents an attractive antimicrobial for immunocompromized patients who need the integrity of their intestinal anaerobic flora for colonization resistance.

Adult↗