Search

A hematologist receives a call from a maternal-fetal medicine (MFM) physician about a previously healthy patient who became ill at 25 weeks’ gestation. Her mental status is deteriorating. There are signs of fetal distress. Platelet count and hemoglobin are falling. The MFM physician is considering the hemolysis, elevated liver enzymes and low platelet count (HELLP) syndrome. For the hematologist, everything seems unfamiliar. Our goal is to provide hematologists with the fundamental knowledge required for understanding and managing these patients who become suddenly and seriously ill during pregnancy and in whom thrombocytopenia and microangiopathic hemolytic anemia are part of their presentation.

BACKGROUND Factor X deficiency (known as; Stuart-Prower factor deficiency or F10 deficiency) is a rare inherited bleeding disorder. It affects one per 1 million individuals worldwide. Patients with inherited bleeding disorder reported difficulty in accessing primary dental care either due to their medical diseases or their related barriers. AIM This article aims to identify barriers to oral health as perceived by the patient with factor X deficiency who had been refused treatment by three dentists. A further aim is to provide dentists with the knowledge required to manage patients diagnosed with factor X deficiency. METHODS AND CASE REPORT A 30-year-old male with the inherited, severe factor X deficiency (

Aim Orodental manifestations are commonly presented in Wiskott Aldrich Syndrome (WAS). The purpose of this paper is to report a case of dental management of a 5-year-old male child with WAS before Hematopoietic Stem Cell Transplantation (HSCT). Such patients are more prone to infection due to pretransplantation chemotherapy and posttransplantation immunosuppression; thus, it becomes imperative to eliminate all potential sources of infection before transplantation. Materials and methods Fluctuating blood parameters before the dental procedure was an important challenge in rendering dental treatment. Dental procedures were carried out under general anesthesia by maintaining the hematological parameters with blood and platelet transfusion. The conventional dental treatment may not be applicable in such patients as failure of dental treatment can cause the failure of HSCT, and it has to be modified based on the clinical acumen and recommendations. Conclusions This case report focuses on the measures to be taken before, during, and after the dental procedure to ensure the success of the dental therapy and prevent failure of HSCT due to residual dental foci of infection. A multidisciplinary approach involving a pedodontist, a pediatrician, and a hematologist can improve the quality of life of such patients.

Purpose A considerable barrier to global pediatric oncology efforts has been the scarcity and even absence of trained professionals in many low- and middle-income countries, where the majority of children with cancer reside. In 2013, no dedicated pediatric hematology-oncology (PHO) programs existed in Ethiopia despite the estimated annual incidence of 6000-12000 cases. The Aslan Project initiative was established to fill this gap in order to improve pediatric cancer care in Ethiopia. A major objective was to increase subspecialty PHO-trained physicians who were committed to practicing locally and empowered to lead programmatic development. Methods We designed and implemented a PHO training curriculum to provide a robust educational and clinical experience within the existing resource-constrained environment in Ethiopia. Education relied on visiting PHO faculty, a training attachment abroad, and extraordinary initiative from trainees. Results Four physicians have completed comprehensive PHO subspecialty training based primarily in Ethiopia, and all have remained local. Former fellows are now leading two PHO centers in Ethiopia with a combined capacity of 64 inpatient beds and over 800 new diagnoses per year; an additional former fellow is developing a pediatric cancer program in Nairobi, Kenya. Two fellows currently are in training. Program leadership, teaching, and advocacy are being transitioned to these physicians. Conclusions Despite myriad challenges, a subspecialty PHO training program was successfully implemented in a low-income country. PHO training in Ethiopia is approaching sustainability through human resource development, and is accelerating the growth of dedicated PHO services where none existed 7 years ago.

Hemophilia-a bleeding disorder due to the lack of clotting factors-is mostly induced by genetic factors. Its most common type is hemophilia A. Hemorrhage in hemophilia A may occur in several different sites of the body, including those inside the oral cavity, such as mucous membrane and gum. However, only a few studies and case reports on dental issues of hemophilia A patients have been conducted. In clinical dentistry, treatment procedures are invasive, possibly leading to more severe bleeding, especially in hemophilia A cases. This paper presents the case of a 4-year-old boy diagnosed with severe hemophilia A, who also had several dental problems that needed endodontic treatment. We used laser diode to manage pulpotomy under general anesthesia to treat his dental diseases. His treatment procedure was performed by a dental surgeon, a hematologist, and an anesthesiologist.

Background Children with sickle cell disease (SCD) are at increased risk of death from invasive bacterial infections. Emergent evaluation of fever allows early treatment of potentially fatal infections. Limited data exist regarding caregiver adherence to physician recommendations of prompt medical evaluation of fever in children with SCD. Better understanding of parental behavior around fever management may inform improved models for support in families of children with SCD. Procedure Cross-sectional survey based on health belief domains, Wake Forest trust scales, and self-reported adherence among 163 caregivers of children with SCD during routine hematology visit. Results Fifty-five percent of caregivers were adherent to fever evaluation recommendations as defined by “always” seeking medical evaluation of fever in their child with SCD. Perceived susceptibility to fever/infection, benefits of prompt evaluation, and cues to action were significantly different between those who adhere to recommendations versus those who do not. Twenty-five percent believe their child does not need antibiotics with every fever whereas 17% believe their child does not need evaluation of fever after immunizations. Fifty-seven percent report their employer understands missing work whereas 25% report concern regarding cost of evaluation. Trust in their child's hematologist and medical profession was high (composite scores 23.4/25 and 21/25, respectively). Conclusion Despite a high degree of agreement in importance of fever evaluation and high levels of trust, many caregivers do not consistently seek care when their child has a fever. Future studies should address additional barriers to seeking emergency care in children with SCD and fever. Pediatr Blood Cancer © 2015 Wiley Periodicals, Inc.

Background Free tissue transfers performed in patients with hematological diseases represent significant challenges for micro-surgeons. There are rare literatures that address the outcome in these patients. Therefore, we collected our database, analyzed the outcome, reliability, and related-management of microsurgical technique in the patients with hematological diseases. Methods A retrospective chart review of 20 patients with hematological disorders who received free tissue transfers during 20-years period in a single microsurgical center was done. Eleven patients who received head and neck reconstruction were found to have hyperfibrinogenemia. Seven patients with reactive thrombocytosis after trauma, and two patients with leukemia had soft tissue defects in the upper and lower extremities. Twenty-six flaps were used for free tissue transfers. Intra-operatively all patients received intravenous 5,000 Ud of heparin post immediate reperfusion. Anti-coagulant medication such as Dextran-40 or prostaglandin-E1 (PGE1) was given postoperatively. Results Twenty-three of the 26 free flaps survived without vascular compromise. Intra-operatively all patients received intravenous 5,000 Ud of heparin post immediate reperfusion, and anti-coagulant medication such as Dextran-40 or prostaglandin-E1 was given to the patients postoperatively. The three failed cases were found in patients with hyperfibrinogenemia and needed further reconstruction with another flap. The overall success rate was 88.5% (23/26). Conclusions Hematologic disorder is not a predicted factor of free flap failure. The key factors for success flap survival in patients with hematologic disorders include preoperative knowledge of the medical condition and monitoring potential post-operative complications, aggressive hematologist consultations, and meticulous non-traumatic surgical anastomosis. © 2014 Wiley Periodicals, Inc. Microsurgery 34:505–510, 2014.

Anemia is now recognized as a risk factor for a number of adverse outcomes in the elderly, including hospitalization, morbidity, and mortality. What constitutes appropriate evaluation and management for an elderly patient with anemia, and when to initiate a referral to a hematologist, are significant issues. Attempts to identify suggested hemoglobin levels for blood transfusion therapy have been confounded for elderly patients with their co-morbidities. Since no specific recommended hemoglobin threshold has stood the test of time, prudent transfusion practices to maintain hemoglobin thresholds of 9-10 g/dL in the elderly are indicated, unless or until evidence emerges to indicate otherwise.

Patients with an apparent bleeding disorder can usually be diagnosed by a careful history, physical examination, and screening laboratory tests. However, at times the constellation of bleeding signs and symptoms fail to be explained by test results and/or our current understanding of hemostatic mechanisms. One such patient is the subject of the current report. She is a 13-year-old female with a history of striking bleeding manifestations, including spontaneous hemorrhage from her eyes, scalp, hands, and feet. She was evaluated by one of the authors at a teaching hospital in Mumbai, India in March 2009 during the filming of a National Geographic Channel documentary characterizing puzzling medical disorders encountered in India. Given her unusual bleeding manifestations, she received international media attention at the time. National Geographic and a film company in the United Kingdom subsequently expressed interest in highlighting the patient to document her seemingly rare hematologic disorder and contacted the American Society of Hematology to identify an American hematologist to further investigate the case. With consent of the family and collaboration with a hematologist practicing at a teaching hospital in Mumbai, filming commenced during March 2009 in an attempt to capture the patient's diagnosis and the cultural and medical milieu in which the bleeding events occurred. Am. J. Hematol. 88:713–716, 2013. © 2013 Wiley Periodicals, Inc.

Background Pediatric procedural sedation outside of the operating room is performed by a variety of pediatric specialists. Using the database from the Pediatric Sedation Research Consortium (PSRC), patient demographics, medications used, diagnoses, complications, and procedures involved when pediatricians provided sedation in this cohort, were described. ‘Pediatrician’ was defined as a general pediatrician, cardiologist, endocrinologist, gastroenterologist, hematologist/oncologist, neurologist, pulmonologist or hospitalist. Methods Data were collected by the PSRC, a group of 35 institutions dedicated to improving sedation care for children. Members prospectively enrolled consecutive patients who received sedation or anesthesia for diagnostic or therapeutic procedures. Data on demographics, primary diagnoses, procedures, medications, interventions, and complications were collected and stored on a Web-based data collection tool. Results A total of 12 113 sedations performed by pediatricians were submitted from 1 July 2004 to 31 December 2008, compared to 119 665 cases performed by non-pediatricians. Pediatrician patients were more frequently non-emergency American Society of Anesthesiologists (ASA) class I or II, aged 2–8 years old, with a neurologic primary diagnosis, being sedated for a radiologic procedure with a sedative. Distraction techniques were used more frequently in the pediatrician group (11.9% vs 3.1%). The most common complication encountered was inadequate sedation, which occurred 2.2% of the time. Conclusions Pediatricians sedate for a variety of patients within the PSRC, but the patients tended to be younger, predominately ASA class I or II, non-emergency, and undergoing non-painful procedures when compared to non-pediatrician providers. The patient demographics, medications used, diagnoses, complications, and procedures involved varied between the groups significantly. Complication rates were similar between the groups.

The purpose of this cross-sectional study was to assess and compare the oral health of children with neutropenia, who are under the active care of a hematologist in a designated marrow failure and myelodysplasia program, to a healthy control group. Children aged 6-18 with neutropenia attending the Marrow Failure and Myelodysplasia Program at SickKids Hospital and controls attending the Children's Clinic, Faculty of Dentistry, University of Toronto were asked to participate in the study consisting of a patient questionnaire followed by a dental and radiographic examination. Fifteen patients with neutropenia (mean age 12.14 ± 4.04 years) and 26 healthy controls (mean age 11.61 ± 3.82 years) participated in this study. Patients with neutropenia reported significantly increased mouth sores (p < .008) and bleeding gums while brushing (p < .001). The dmft/t score was significantly lower for the neutropenia group (p < .009). The clinical examination also showed that there were no statistically significant differences with respect to ulcerations, gingival recession, tooth mobility, gingival inflammation, periodontal bone loss, DMFT/T scores, plaque, and calculus levels. Preliminary data demonstrates that pediatric patients who are under the active care of a hematologist do not present with an increased risk of oral diseases.

Systemic bleeding at the time of postpartum hemorrhage (PPH) is usually the result of coagulopathy that has developed acutely as a result of massive hemorrhage after uterotonics and sutures have failed. Occasionally, the patient has a preexisting coagulopathy, but more often, coagulopathy arises acutely as the result of massive hemorrhage, which is usually related to obstetrical and less often surgical bleeding. Despite being able to identify risk factors for PPH in the antenatal and intrapartum period, the majority of women who ultimately develop PPH do not have any such factors and every pregnancy is at risk. The coagulopathy associated with massive PPH may be due to hemodilution, failure of liver synthetic function as occurs with acute liver failure of pregnancy, or disseminated intravascular coagulation (DIC). There are no data from clinical trials to help guide management of transfusion in PPH, although the management of blood component therapy in severe PPH is similar to that in other massive hemorrhage. Standard practice is to replace fibrinogen to maintain a level of ≥ 100 mg/dL, yet recent evidence suggests that the level of fibrinogen needed to prevent PPH is at least 400 mg/dL. Recombinant activated factor VIIa (rFVIIa) has been used in the management of severe PPH unresponsive to blood component therapy. Coagulation laboratory evaluation may be useful in guiding hemostatic management during massive PPH, but for the results to be useful, they must be rapidly available and provide information that would not be available from clinical assessment alone. The hematologist or hemostasis expert has the opportunity to make the difference between life and death for the patient experiencing massive PPH.

Background Sickle cell disease (SCD) requires coordinated ambulatory care from generalists and hematologists. We examined when children with SCD establish ambulatory care connections, whether these connections are maintained, and how these connections are used before and after hospitalizations. Procedure We conducted a retrospective cohort study of Medicaid-insured Maryland children with SCD from 2002 to 2008. For children enrolled from birth, time to first, second, and third generalist and first hematologist visits was plotted. For all children, we analyzed ambulatory visits by age group, by emergency department (ED) and hospital use, and before and after hospitalizations. Results The overall study cohort comprised 851 children; 178 provided data from birth. Ambulatory care connections to generalists were made rapidly; connections to hematologists occurred more slowly, if at all (38% of children had not seen a hematologist by age 2 years). Visits with generalists decreased as patients aged, as did visits with hematologists (54% of children in the 12–17 year age group had no hematology visits in 2 years). Children with higher numbers of ED visits or hospitalizations also had higher numbers of ambulatory visits (generalist and hematologist). Most children had visits with neither generalists nor hematologists in the 30 days before and after hospitalizations. Conclusions Medicaid-insured children with SCD rapidly connect with generalists after birth; connections to hematologists occur more slowly. The observation that connections to generalists and hematologists diminish with time and are infrequently used around hospitalizations suggests that the ambulatory care of many Medicaid-insured children with SCD may be inadequate. Pediatr Blood Cancer 2012; 59: 888–894. © 2012 Wiley Periodicals, Inc.

BACKGROUND: The impact of physicians' disease-specific expertise on patient outcome is unknown. Although previous studies suggest a survival advantage for cancer patients cared for at high-volume centers, these observations may simply reflect referral bias or better access to advanced technologies, clinical trials, and multidisciplinary support at large centers. METHODS: We evaluated time to first treatment (TTFT) and overall survival (OS) of patients with newly diagnosed chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL) at a single academic center based on whether they were cared for by a hematologist/oncologist who subspecializes in CLL (CLL hematologist) or a hematologist/oncologist with expertise in other areas (non-CLL hematologist). RESULTS: Among 1309 newly diagnosed patients with CLL cared for between 1999 and 2009, 773(59%) were cared for by CLL hematologists and 536 were cared for by non-CLL hematologists. Among early-stage patients (Rai 0-I), median TTFT (9.2 vs 6.1 years; P < .001) and OS (10.5 years vs 8.8 years; P < .001) were longer for patients cared for by CLL hematologists. For all patients, OS was superior for patients cared for by CLL hematologists (10.5 years vs 8.4 years; P = .001). Physician's disease-specific expertise remained an independent predictor of OS after adjusting for age, sex, stage, and lymphocyte count at diagnosis. Patients seen by a CLL hematologist were also more likely to participate in clinical trials (48% vs 16%; P < .001). CONCLUSIONS: Physician disease-specific expertise appears to influence outcome in patients with CLL. To the greatest extent possible, patients should be cared for by a hematologist/oncologist expert in the care of their specific malignancy. When not possible, practice guidelines developed by disease-specific experts should be followed. Cancer 2012. © 2011 American Cancer Society.

As recently as the 1970s, children born with sickle cell disease (SCD) were unlikely to survive into adulthood. With advances in medical care, most patients now survive childhood and live into their forties or beyond [1,2]. A better transition from pediatric- to adult-focused care is therefore increasingly important [3]. Despite recent awareness of the importance of transition by the SCD community, little is known about existing transition programs. We conducted a survey of pediatric sickle cell clinics to describe current transition practices and identify areas for improvement. Survey topics included program demographics, transition logistics, assessment of patient transition readiness and independence, transition preparation, and program evaluation. Twenty-three clinics (77%) report having a transition program, although half have been in place for under 2 years. There is wide variation in specific transition practices. Most centers (97%) have an identified accepting adult provider, however, only 60% routinely transfer their patients to an adult hematologist specializing in SCD. Although there has been a recent effort to establish transition programs in pediatric sickle cell clinics, specific practices vary widely. As anticipated, lack of an accepting adult hematologist with an interest in SCD emerged as a common barrier to transition.

An array of monogenic immune defects marked by autoimmunity, lymphoproliferation, and hyperinflammation rather than infections have been described. Primary immune regulatory disorders pose a challenge to pediatric hematologists and oncologists. This paper focuses on primary immune regulatory disorders including autoimmune lymphoproliferative syndrome (ALPS) and ALPS-like syndromes, immunodysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) and IPEX-like disorders, common variable immunodeficiency (CVID), CVID-like, and late-onset combined immunodeficiency (CID) disorders. Hyperinflammatory disorders and those associated with increased susceptibility to lymphoid malignancies are also discussed. Using a case-based approach, a review of clinical pearls germane to the clinical and laboratory evaluation as well as the treatment of these disorders is provided.

Objectives: The purpose of this study was to examine hematologist and patient perspectives about the side-effects of the corticosteroid treatment of immune thrombocytopenic purpura (ITP) and their perspectives about the patient’s risk for bleeding. The specific aim was to compare patient and hematologist perspectives and, if a difference was documented, the implications of that difference. We hypothesized that patients with ITP may have more concern about corticosteroid side-effects and less concern about serious bleeding than hematologists. Methods: We surveyed 80 patients in the Oklahoma ITP Registry and all 83 hematologists in Oklahoma about the occurrence and severity of 18 corticosteroid side-effects and risks for serious bleeding. Results: Response rates were 80% (patients) and 71% (hematologists). Responses of patients and hematologists were significantly different from each other regarding both the frequency of severe corticosteroid side-effects and the risk of serious bleeding. For 13 of the 18 corticosteroid side-effects, patients reported more frequent occurrence of severe symptoms than hematologists (P

An understanding of variations in the delivery and outcomes of clinical care by physician specialty can be used to target education and interventions to the most relevant professional societies and training programs. The assessment of these variations, however, depends on the quality of the data used to measure physician specialty. The Surveillance, Epidemiology, and End Results (SEER)-Medicare dataset, which consists of cancer registry data linked to Medicare enrollment and claims files, contains physician specialty on the claims and is commonly used by health services researchers to examine specialty-related variations in care of people with cancer (Earle et al. 2002, 2006; Keating et al. 2003; National Cancer Institute 2007). Information about physician specialty available on Medicare claims can be supplemented by linking to sources such as the Unique Physician Identification Number (UPIN) Registry or the American Medical Association (AMA) Physician Masterfile (AMA 2004; NHIC 2006). Baldwin et al. (2002) reviewed the availability and quality of data on physician characteristics in Medicare claims and found that while these data are useful, investigators must understand the limitations of data on physician specialty so that they might decide the best approach to a particular research question and how these limitations may affect findings. For example, Rosenblatt and colleagues found that the AMA Masterfile identified 97 hematologist/oncologists in Washington state in addition to the 58 hematologist/oncologists found in that state's Medicare claims, a 167 percent increase (Rosenblatt et al. 1998; Baldwin et al. 2002). Thus, researchers investigating the role of hematologist/oncologists potentially would underestimate the number of physicians with this specialty if using Medicare claims alone. The extent to which there is agreement on cancer-specific physician specialty variables between Medicare claims, the UPIN Registry, and the AMA Masterfile for the entire SEER-Medicare dataset is unknown. Given the additional time and financial investment that is required to obtain and link the UPIN Registry and/or the AMA Masterfile to SEER-Medicare data, we believe a more complete understanding of the agreement on cancer-specific physician specialty between these data sources would be useful. Such information could be used by future investigators to determine whether seeking additional data on specialty will enhance the quality of their cancer-related research. The purpose of this paper is to compare the agreement and identification of physicians by cancer specialty between SEER-Medicare claims, the UPIN Registry, and the AMA Masterfile. We also demonstrate an approach to refine the determination of physicians as cancer specialists when using all three data sources, and we provide an overview of Medicare claims-based information on physician specialty, including the process of obtaining the data.

Spontaneous gingival bleeding can occasionally be the only sign of systemic bleeding problems. The diagnosis and management of such conditions may challenge the skills of both the hematologist and the oral physician. We present this patient because of several confusing phenomena that were encountered: unprovoked periodontal hemorrhage, which endangered the life of an otherwise asymptomatic young adult male; (especially unusual was a platelet count above 150,000 cells per microliter of blood), and presentation of idiopathic thrombocytopenia purpura through spontaneous periodontal hemorrhage alone. This case history also highlights the fact that medical intervention to correct the underlying aberration of hemostasis is necessary for local dental measures to successfully stop bleeding. In contrast with the definition of thrombocytopenia, the "within normal" count of platelets should not exclude the possibility of idiopathic thrombocytopenia purpura; a fact that, if ignored, can make the diagnosis and management of bleeding troublesome.

Vascular anomalies (VAs) are classified as tumors or malformations depending on their clinical characteristics, pathological diagnosis, and genomic information. Diagnosis can be challenging because of the heterogeneity of clinical presentation; thus, the best diagnosis and care are provided by an interdisciplinary team of specialists. Over the past 10 years, an increasing number of pediatric hematologist/oncologists are caring for patients with VAs secondary to new medical therapy options and clinical trials. This paper focuses on complicated VA issues often seen by the pediatric hematologist/oncologist. The paper reviews clinical pearls on diagnosis, histology, radiology, and treatment options.

To sum up, some hematological side effects are seen after antihypertensive drugs. They are, however, few, usually not severe and furthermore reversible. And so, from the viewpoint of the hematologist, there are no contraindications against antihypertensive treatment. The prevalence of side effects on blood and blood-forming organs from antihypertensive drugs is low. Of certain interest are 1) the tendency of modern diuretics (thiazides) to cause a moderate and reversible thrombocytopenia and 2) the positive antiglobulin reaction (Coomb's test) sometimes seen after alpha-methyldopa therapy and in a few cases causing autoimmune hemolytic anemia. Even though case reports have appeared on leukopenia/agranulocytosis related to almost all drugs used in the treatment of hypertension, none of these drugs seem to be especially prone to cause any damage to leukocytes and/or leukocyte production.

Purpuras include a wide spectrum of cutaneous disorders characterized by extravasation of red blood cells into the skin with consequent release of hemoglobin. Various other pigments deriving from heme are subsequently found into the skin within 2–3 weeks, accounting for the color changes (purple, orange, brown, yellowish, green-blue) which may occur in most purpuric lesions. Too often the factors leading to these disturbances are obscure. Sometimes they are obscure mainly to the dermato-venereologist as they are generally considered more pertinent to the field of interest of other specialists, i.e. in hematology or internal medicine. The dermato-venereologist should be familiar with these cutaneous conditions and, when necessary, cooperate with the hematologist in order to evaluate the cutaneous and extracutaneous signs and symptoms and to schedule the proper systemic and/or topical therapies. Learning objective. At the conclusion of this learning activity, participants should be able to discuss the clinical and histological presentations of purpuric disorders and know which tests should be done to allow proper diagnosis and treatment. The participants should also be aware of the controversies concerning the pathogenesis of some kinds of purpuras (i.e. palpable purpuras), of the evolution of terminology and finally of the different therapeutic options and regimens.

The hepatitis C virus (HCV) has been cloned, and assays capable of detecting antibody to HCV recombinant proteins (anti-HCV) have been developed. Concurrent with the cloning and development of the anti-HCV screening tests, trials with interferon alfa-2b have documented biochemical and histologic improvement in the indices of hepatitis C and non-A, non-B (NANB) in patients who were chronically infected. Subsequently, the anti-HCV assays and interferon alfa-2b have become clinically available. These new detection and treatment modalities can now be used in the management of hemophilics and other chronically transfused patients who bear great risk for blood-borne infections and manifest signs of chronic hepatitis. © 1993 Wiley-Liss, Inc.

Optimum care of patients with hemophilia requires cooperation among the hematologist, the surgeon, nurses, and blood bank personnel. Relatively normal hemostasis can be achieved and maintained in factor-deficient individuals in the perioperative period. Surgical procedures in patients with hemophilia can be accomplished safely with careful monitoring of coagulation factor levels and appropriate replacement therapy.

The activated partial thromboplastin time (aPTT) is frequently used to assess overall competency of the intrinsic pathway of coagulation. An abnormal value may be caused by any of several abnormalities along this pathway or by many other variables including the presence of inhibitors, poor collection of the sample, or variables in the laboratory. When the cause for the prolongation is unknown to the requesting physician, the hematologist may be consulted. In this prospective study, the cause and perceived hemostatic risk to 100 consecutive patients referred to use for consultation regarding a prolonged aPTT of previous unknown cause were evaluated. We found that these abnormal aPTTs may be either indicative of a hemostatic defect, in 50% of the cases, or of no particular risk, in 36% of the cases. In 14%, the aPTT was artifactually prolonged. Most (81%) patients with a prolonged aPTT due to a hemostatic defect had an abnormal hemostatic history but some (19%) did not. Even among true abnormal tests, the degree of abnormality indicated little or nothing about hemostatic competency. We conclude that the cause of an abnormal aPTT is more important than the result itself. These data may be of use to those who consult on such matters.

Paroxysmal nocturnal hemoglobinuria (PNH) is an uncommon blood stem cell disorder. The acquired defect is in the cell membrane of the precursor of the affected clone, which renders erythrocytes, platelets and granulocytes unusually sensitive to the hemolytic effects of complement. It is characterized by chronic hemolysis, intermittent hemoglobinuria, thrombotic events and bone marrow hypoplasia. Hemolysis is precipitated by infection, strenuous exercise, surgery or menstruation. The dilemma of managing a PNH patient is that both the periodontal infection and the surgical treatment are precipitating factors. Management is further complicated by the fact that the patient is receiving corticosteroids. The treatment of the PNH patient is complex and potentially hazardous. It requires close cooperation between the periodontist and the hematologist. This paper describes the management of one such case.

Patients with hemostatic disorders about to undergo surgery present a special challenge for the otolaryngologist. The most common coagulation disorders constituting more than 90% of all patients seen with severe inherited bleeding defects are hemophilia and von Willebrand's disease (pseudohemophilia). Neither of these is an absolute contraindication for surgery. Hemophilia, a sex-linked disorder, can be divided into two distinct classes: hemophilia A (classic hemophilia), a deficiency of factor VIII production, and hemophilia B (Christmas disease), a deficiency of factor IX production. Von Willebrand's disease is an autosomal dominant disorder consisting of a decrease in factor VIII activity plus a prolonged bleeding time and abnormal platelet aggregation with ristocetin. Careful preoperative evaluation and early hospitalization are important for these patients. The close cooperation of a hematologist, monitoring availability and proper replacement of these factors, is essential. Careful postoperative attention should be directed at the maintenance of sufficient therapeutic levels of replacement therapy for a minimum of 7–10 days. We shall present cases of patients with inherited bleeding diatheses who underwent successful surgery at our institution. The use of Amicar® and Avitene® will also be presented.