Laryngeal mucous membrane plasmacytosis with 15 year follow-up: Case report and literature review.

Mucous membrane plasmacytosis (MMP) is an uncommon variant of mucositis represented by a polyclonal plasma cell infiltration of mucosal tissue. Various clinical presentations in the upper airway have been reported ranging from erythematous mucosa to fungating masses. Histologic features include mucosal epithelial hyperplasia or psoriasiform changes with a dense submucosal infiltrate of polytypic plasma cells.

Physiological measurements corroborate symptomatic improvement after therapeutic leukapheresis in a pregnant woman with chronic myelogenous leukemia.

Therapeutic leukapheresis can control the white blood cell count (WBC) of pregnant women with chronic myelogenous leukemia (CML) who have hyperleukocytosis without leukostasis. The medical justification for this treatment has not been objectively documented. We report a 27-year-old woman, diagnosed with CML at 10-week gestation, who developed severe dyspnea on exertion.

Characterization of the anti-factor VIII immunoglobulin profile in patients with hemophilia A by use of a fluorescence-based immunoassay.

Background: The development of neutralizing antibodies, referred to as inhibitors, against factor VIII is a major complication associated with FVIII infusion therapy for the treatment of hemophilia A (HA). Previous studies have shown that a subset of HA patients and a low percentage of healthy individuals harbor non-neutralizing anti-FVIII antibodies that do not elicit the clinical manifestations associated with inhibitor development.

Objective: To assess HA patients' anti-FVIII antibody profiles as potential predictors of clinical outcomes.

Chronic basophilic leukemia: a rare form of chronic myeloproliferative neoplasm.

Chronic basophilic leukemia is a rare and poorly characterized entity. Only a limited number of cases have been described. Herein, we report a patient who presented with fatigue, weight loss, leukocytosis, persistent prominent basophilia, and mild eosinophilia.

Home-based factor infusion therapy and hospitalization for bleeding complications among males with haemophilia.

Information from the medical records of 2650 US males with haemophilia living in six states was used to examine the influence of infusing factor concentrate at home (home therapy) and other variables on rates of hospitalization for a haemorrhagic bleeding complication (HBC) over a 4-year period. Bleeding complications included actual and suspected haemorrhagic events but excluded elective admissions for procedures necessitated by haemorrhage (e.g.

Long-term nonprogressive infection with human immunodeficiency virus type 1 in a hemophilia cohort.

Seven long-term nonprogressors (LTNPs) have been identified in a cohort of 128 human immunodeficiency virus (HIV)-1 infected individuals with hemophilia. Studies included quantitation of virus by polymerase chain reaction, characterization of primary virus isolates in vitro, analysis of lymphocyte surface markers, and measurement of virus-specific cytotoxic T lymphocytes (CTLs). Viruses of LTNPs exhibited slow growth in vivo and in vitro.

Human immunodeficiency virus in semen arises from a genetically distinct virus reservoir.

The reservoir of human immunodeficiency virus (HIV) in semen is unknown. Several lines of evidence suggest that semen HIV may not arise from the same reservoir of infection as peripheral blood. If true, the viral burden in the two compartments could be qualitatively and quantitatively different, a scenario of potentially profound significance for the design of effective strategies of treatment, disease monitoring, and infection containment.

Human immunodeficiency virus type 1-specific cytotoxic T lymphocytes (CTL), virus load, and CD4 T cell loss: evidence supporting a protective role for CTL in vivo.

The relationships between primary human immunodeficiency virus type 1 (HIV-1) Gag-specific cytotoxic T lymphocyte (CTL) frequency, virus load, and CD4 T cell loss were evaluated in a group of 46 HIV-1-infected persons with hemophilia. Freshly isolated peripheral blood mononuclear cells in limiting dilution assays were used to measure HIV-1 Gag-specific CTL frequencies. Concurrent measurements of virus load and lymphocyte surface markers were obtained.

DDI and liver tests in HIV-1 seropositive haemophiliacs.

Many patients with haemophilia who are HIV-1 seropositive are co-infected with the hepatitis C virus with variable degrees of underlying liver disease. To evaluate whether the use of the antiretroviral agent Dideoxyinosine (DDI) causes worsening of hepatic dysfunction as measured by liver enzyme tests, we reviewed our cohort of patients previously treated with monotherapy with Zidovudine (AZT) and subsequently changed to DDI. Seventeen patients (median age: 34 years, median absolute CD4 lymphocyte cell count: 86 cells μL(-1) ) were included in this study.

AIDS-associated non-Hodgkin's lymphomas as primary and secondary AIDS diagnoses in hemophiliacs. Hemophilia Malignancy Study Group.

We studied the characteristics and temporal trends of AIDS- associated non-Hodgkin's lymphoma (AIDS-NHL) in individuals with hemophilia. Prospective data were collected on 33 HIV-positive hemophiliacs with AIDS-NHL enrolled in the Hemophilia Malignancy Study (HMS), of whom 21 had primary and 12 had secondary or subsequent AIDS-defining illnesses, and analyzed for frequency and temporal trends. As compared with primary AIDS- NHL, secondary AIDS-NHL occurred at an older mean age, 37 versus 29 years (p = 0.

Hemostasis: a practical review of conservative and operative care.

Progress in the treatment of persons with hemophilia has improved quality of life and made surgical intervention in these patients possible. Optimum management should include a facility equipped with a special coagulation laboratory, a hematologist proficient in the management of coagulation disorders, a surgeon with experience operating on persons with a clotting disorder, and a blood bank with experienced personnel. Persons with inhibitors should undergo elective procedures only after consultation between surgeon and hematologist.

Inhibitors in congenital haemophilia.

Inhibitor formation is a serious complication, occurring in 24-52% of patients with haemophilia A and in 1.5-3% of patients with haemophilia B. Low-titre inhibitors can easily be overcome and do not represent a treatment challenge.

Hepatitis C seropositivity in HIV-negative children with severe haemophilia.

With the advent of new viral inactivation and purification methods for factor concentrates in the 1980s, transmission of both HIV-1 and hepatitis viruses has been significantly decreased. However, on routine annual testing of the paediatric population at the New England Hemophilia Center (NEHC), several children were noted to be hepatitis C (HCV) seropositive. Thus, a retrospective review of children with severe haemophilia was undertaken.

Anaphylaxis after treatment with recombinant factor VIII.

Background: Treatment of hemophilia patients with recombinant factor VIII concentrates has not previously been associated with anaphylaxis.

Study Design And Methods: A 5-week-old boy with severe hemophilia A developed dyspnea, cyanosis, hypotension, and a diffuse urticarial rash following treatment with a recombinant factor VIII (Recombinate). To identify the cause of anaphylaxis in this patient, the vial lot was examined for the presence of endotoxin, and a checkerboard immunoblotting technique was used to test serum and/or plasma samples from the patient and mother for the presence of antibodies (IgA, IgG, IgE, and IgM) to Recombinate-related antigens (recombinant factor VIII, von Willebrand factor, human serum albumin, Chinese hamster ovary proteins, bovine serum albumin, mouse monoclonal anti-human factor VIII, polyethylene glycol 3350), and to ethylene oxide, the agent used to sterilize the infusion equipment.

The knowledge and perception of factor concentrate in persons with haemophilia A.

Unlabelled: Objectiues. To evaluate haemophilia patients' knowledge and understanding of factor concentrate products and to assess their feelings regarding purity and viral safety of these products.

Methods: Sixty questionnaires were distributed and completed by patients or parents of patients with severe/ moderate haemophilia A at two large comprehensive haemophilia centres in the USA and Italy.

Tibial bone plug resorption with extra-articular cyst: a rare complication of anterior cruciate ligament reconstruction.

Anterior cruciate ligament (ACL) reconstruction failure resulted in tibial bone plug resorption and the formation of a large extra-articular cyst. To our knowledge, this is the first report of this kind of ACL failure. The relationship to known factors is discussed.

Recombinant coagulation factor products.

There are numerous new coagulation factor concentrates that are being manufactured using recombinant technology. Some are available for use currently for the treatment of bleeding disorders and others are in clinical trials. Recombinant factor VIII concentrates are licenced in most countries and one FVIII concentrate with the B domain deleted should be available in the near future.