Hereditary Hemorrhagic Disorders Research Articles

Epidemiological study of hereditary hemorrhagic disorders in Najaf province, Iraq.

Hemophilia and Von Willbrand disease (VWD) are the most well known types of hereditary hemorrhagic disorders (HHD). Hemophilia affects about 200 000 people worldwide, while VWD affects about 80 000. Because there is a scarcity of epidemiologic studies on hemophilia in Iraq, this study was carried out to evaluate the prevalence and incidence trends, as well as to identify some clinical and epidemiological features of hemophilia patients in Najaf province, Iraq. This study was carried out in the Najaf's hemophilia center. The data were obtained by reviewing all patients' documents, as well as the center registration book from 2011 to 2021. In addition, the Ministry of Health provided relevant population data for Najaf. Notably, there are currently 214 patients registered in Najaf province. The results revealed that the severe form of hemophilia A was the permanent type of HHDs in the patients compared with the rest of the types that include HHD with no significant difference Pat least 0.05. The frequency of this group of disorders appeared to increase in the period between 2011 and 2013, especially in 2012 followed by a decline in the incidence until 2021, which recorded a sudden increase in these disorders. These findings highlight that hemophilia types A and B were the most prevalent disorders of HHD in Najaf province, and the increase in number of newly recorded cases because of consanguineous marriage increased recently in this area.

Gray Platelet Syndrome-Unusual Presentation with Spontaneous Splenic Rupture: A Case Report and Literature Review.

Gray platelet syndrome (GPS) is a rare hereditary hemorrhagic disorder characterized by macrothrombocytopenia and the absence of alpha-granules in platelets. Clinically, mild-to-moderate bleeding is the main manifestation, often accompanied by thrombocytopenia, splenomegaly, and myelofibrosis. Here, we present a case of a 15-year-old male patient with a history of hepatosplenomegaly, and thrombocytopenia for 8 years, who presented with sudden generalized abdominal pain. Despite initial suspicion of gastroenteritis, diagnostic imaging revealed an extensive hemoperitoneum. Subsequent genetic testing confirmed the diagnosis of GPS, which had not been previously identified. This case highlights the importance of considering inherited platelet disorders should be considered in adolescents with long-standing thrombocytopenia, and emphasizes the need for thorough evaluation in patients with suggestive symptoms.

Budget Impact Analysis of the use of emicizumab for bleeding prophylaxis in patients with Hemophilia A with inhibitors in the Brazilian Health System

Hemophilia A is a hereditary hemorrhagic disorder characterized by deficiency or dysfunction of the coagulation protein factor VIII. The standard therapy consists of factor VIII replacement, which can lead to the development of neutralizing antibodies (inhibitors), a major complication that implies a burden on patient health and the healthcare system because of the decreased quality of life and further costs with treatment. The current work was to assess the budget impact of a bispecific monoclonal antibody, for patients with inhibitors, considering the Brazilian Public System perspective. Results demonstrated high efficacy on preventing bleeds and savings of R$ 1.5 billion in five years.

Using Whole Exome Sequencing to Identify the Pathogenic Variant of F7 C.681+1 G>t As a Potential Cause of Severe Bleeding Clinic Presentation in a Newborn Baby

Ching-Tien Peng1, 2, 3 Chien-Yu Lin4 Yin-Ting Chen1 1.Division of Pediatric Hematology & Oncology, China Medical University Children's Hospital, Taichung, 40447, Taiwan 2.Department of Biotechnology, Asia University, Taichung, 40447, Taiwan 3.Divisions of Pediatrics, China Medical University Hsinchu Hospital, Zhubei City, Hsinchu County, 30272, Taiwan. 4.Divisions of Laboratory Medicine, China Medical University Hsinchu Hospital, Zhubei City, Hsinchu County, 30272, Taiwan. Objective: The clinical manifestations of Factor VII deficiency vary, most cases remain asymptomatic or mild, and cases with severe clinical presentation are rarely reported. It is a rare hereditary hemorrhagic disorder passed on through autosomal recessive inheritance. We present a 2-day-old male infant with large cephalohematoma and intracranial hemorrhage, who was diagnosed using whole exome sequencing with Factor VII deficiency. Materials and Methods: Whole exome sequencing and Sanger sequencing were used to verify related variants. Other routine blood coagulation and factor activity tests were conducted by Divisions of Laboratory Medicine, China Medical University Hospital, Taiwan. Results: A 2-day-old male newborn was identified to develop left intracranial hemorrhage with unknown causes. The laboratory coagulation factor test results indicated a prolonged prothrombin time and normal activated partial thromboplastin time. The test results for Fibrinogen (279.9 mg/dL) and Factor activity analysis, included Factor VIII (85.6%), Factor V (49.3%), Factor VII (<1.0%), Factor IX (81.3%), and Factor X (42.7%). The results for VWF activity and antigen are normal. Subsequently, whole exome sequencing was used to conduct genetic tests, which revealed the homozygote mutation at F7 c.681+1 G>T (NM_000131). Further variant tests on the family members suggested that the father, mother, and older sister had heterozygote mutation at the same site. Conclusions: We used next-generation sequencing, which uses array-based sequencing to process reactions in parallel and enables the simultaneous investigation of multiple genes at a manageable cost, to identify the variants. While there is still little research on whether there is a relationship between different gene mutations and clinical presentations. The sequencing results revealed homozygote mutation at F7 c.681+1 G>T (NM_000131). The pathogenicity level was indicated to be "Highly pathogenic" in the ClinVar archive. Studies have reported the association of such mutation with severe hemorrhage disorders. Therefore, the present study offers critical empirical insights in that it applies to clinical practice in a clinical setting and identifies a pathogenic variant. We will present the hypothesis mechanism at the ASH meetings.

Papillary thyroid cancer in a child with hemophilia A

Hemophilia A is a hereditary hemorrhagic disorder associated with bleeding episodes and characterized by deficiency or dysfunction of coagulation protein factor VIII. Cancer incidence was found to increase in hemophilic patients. A case of a 6-year old boy with papillary thyroid cancer and hemophilia who underwent a successful total thyroidectomy and bilateral modified radical lymph node dissection with appropriate peri-operative management consisting of Factor VIII replacement is presented.

Spinal Subdural Hematoma in a Young Adult with Hemophilia A: A Case Report

Hemophilia is a set of hereditary hemorrhagic disorders of coagulation-dysfunction that can cause repeated bleeding in various tissues and organs. And the patient can be disabled due to bleeding from bones, joints, and soft tissue, or die due to splenic rupture or intracranial bleeding. So it is crucial to detect the site of bleeding in hemophilia patients quickly. Spontaneous spinal canal hematoma is a rare complication of hemophilia, with less than 50 cases reported to date. It can be easily missed and cause serious harm to patients. Hematoma, if untreated, causes irreversible nerve damage by compression of the spinal cord. Early recognition and treatment are most useful to prevent spontaneous spinal canal hematoma. We describe the case of a 21-year-old man with severe lumbago for two days, who had hemophilia A for a decade. The patient was diagnosed with spinal subdural hematoma by magnetic resonance imaging (MRI) after ruling out other causes of lumbago and treated with drugs by a neurologist. The hematoma showed absorption by MRI after six months. In conclusion, spontaneous spinal canal hematoma is a severe complication of hemophilia, which is often missed. This successful case confirms the importance of early diagnosis and treatment of spontaneous spinal canal hematoma. We hope to increase the clinicians’ vigilance of this disease.

Impact of Blood Groups on Clinical Outcomes in Patients with Von Willebrand Disease

INTRODUCTIONVon Willebrand Disease (vWD) is the most common hereditary hemorrhagic disorder with diverse clinical and genetic characteristics. Transmitted as an autosomal dominant trait (except Type III), it is caused primarily by the quantitative or qualitative deficiency of von Willebrand factor (vWF). ABO blood group types can affect the pathology, severity and hence the presentation of this clinically heterogeneous entity as the genes responsible for ABO blood groups also affects vWF levels in the plasma. We conducted a study to analyze and compare different clinico-demographic aspects among O and non-O blood group patients with Von Willebrand Disease. Our primary aim was to determine the relationship of different blood groups with venous thromboembolism (VTE) in patient with vWD. Our secondary aim was to determine the association of blood groups with different comorbid conditions such as coronary artery disease, cerebrovascular disease, peripheral vascular disease, chronic liver disease, chronic kidney disease etc. in patients with vWD.METHODSA retrospective review was carried out to include all patients diagnosed with Von Willebrand Disease from year 2002 to 2012. Patient population was obtained by using International Classification of Diseases, 9th revision (ICD9) codes. Data was collected with the help of electronic medical records. Multiple clinical variables and demographic characteristics of Von Willebrand Disease patients (n=381) were analyzed and compared between those with type O blood group (205 patients; 54%) and those with type non-O blood groups (176 patients; 46%). Univariate logistic regression was used to obtain crude odds ratios. Predictors with statistically significant p-values were included in a multivariable logistic regression model in order to yield adjusted odds ratio. Statistical significance was set at p<0.05RESULTSPatients with O blood groups were less likely to develop VTE (OR 0.29, 95% CI 0.16-0.52, p < 0.001) as compared to Non-O blood groups in which the prevalence of VTE was much higher (25% vs. 9%). Additionally, patients with O blood groups had relatively lower factor 8 activity (125.9±90.8%; OR 0.99, 95% CI 0.99-1.00, p = 0.018) when compared with Non-O blood groups (168.5±120.6%). Patients with O-blood group had significant, life threatening bleeds at an early age (40.4±20.8 years; OR 0.99, 95% CI 0.97-1.00, p = 0.008) when compared with patients with type non-O blood group (Age 47.1±21.9 years). Additionally, O-Blood groups were related to a lesser prevalence of cerebrovascular disease (OR 0.48, 95% CI 0.29-0.79, p = 0.004) and peripheral vascular disease (OR 0.47, 95% CI 0.25-0.87, p = 0.015) as compared to Non-O blood groups.DISCUSSIONOur study consolidates the fact that vWD patients with O blood group have a lesser risk of developing VTE as compared to non-O blood group patients. Additionally, strokes and peripheral vascular disease is also seen less frequently in O blood groups in patients with Von Willebrand disease. These results suggest that the genes responsible for ABO blood groups also have a significant effect on clinical outcomes in patients with vWD. There are multiple proposed mechanisms to explain this association including presence of ABO antigens on several platelet glycoproteins and glycosphingolipids as well as on the surface of vascular endothelium. More sophisticated, large-scale studies are needed to strengthen the associations we observed in our retrospective study. DisclosuresKuriakose:Kedrion: Speakers Bureau.

Experience of Preimplantation Genetic Diagnosis for Hemophilia at the University Hospital Virgen Del Rocío in Spain: Technical and Clinical Overview.

Hemophilia A and B are the most common hereditary hemorrhagic disorders, with an X-linked mode of inheritance. Reproductive options for the families affected with hemophilia, aiming at the prevention of the birth of children with severe coagulation disorders, include preimplantation genetic diagnosis (PGD). Here we present the results of our PGD Program applied to hemophilia, at the Department of Genetics, Reproduction and Fetal Medicine of the University Hospital Virgen del Rocío in Seville. A total of 34 couples have been included in our program since 2005 (30 for hemophilia A and 4 for hemophilia B). Overall, 60 cycles were performed, providing a total of 508 embryos. The overall percentage of transfers per cycle was 81.7% and the live birth rate per cycle ranged from 10.3 to 24.1% depending on the methodological approach applied. Although PGD for hemophilia can be focused on gender selection of female embryos, our results demonstrate that methodological approaches that allow the diagnosis of the hemophilia status of every embryo have notorious advantages. Our PGD Program resulted in the birth of 12 healthy babies for 10 out of the 34 couples (29.4%), constituting a relevant achievement for the Spanish Public Health System within the field of haematological disorders.

Upper gastrointestinal bleedings in patients with hereditary coagulation disorders in Northwest of Iran

Upper gastrointestinal (UGI) bleeding is one of the most life-threatening complications, in up to 25% of persons with hemophilia (PWH). Recurrent bleeding is common and can be caused by the Helicobacter pylori infection. Our aim was to evaluate the role of H. pylori infection in UGI bleeding in PWH. Ninety patients with hereditary bleeding disorders, 30 patients with (group A), and 60 patients without (group B) a history of UGI bleeding episodes were included. The prevalence of H. pylori infection was investigated by stool antigen test, and serum serologic tests including immunoglobulin G and anti-CagA. Among 90 patients (81 men, nine women, mean age 31.30 ± 10.72 years), 66 patients with hemophilia A, 10 patients with hemophilia B, six patients with Von Willebrand disease, five patients with platelet function disorders, and three patients with other factor deficiencies were evaluated. About 46.7% of patients in group A, and 23.3% of patients in group B were anti-CagA-positive (P=0.02), whereas 76.7% of patients in group A and 51.7% of patients in group B had H. pylori immunoglobulin G antibodies (P=0.02). H. pylori antigen in stool was positive in 76.7% in group A and 55% in group B (P=0.03). No statistically significant difference was found between type and severity of diseases and risk of UGI. H. pylori infection should be considered as an important cause of UGI bleeding in PWH. We would recommend stool antigen test as a new and noninvasive screening test for diagnosis of H. pylori infection in all patients with hereditary hemorrhagic disorders.

Upper Gastrointestinal Bleeding In Patients with Haemophilia In Iran: Prevalence of Helicobacter Pylori Infection.

AbstractAbstract 3662 Background:Upper Gastro Intestinal (UGI) Bleeding is one of the most life-threatening complications, and occurs in up to 25% of persons with Hemophilia (PWH).Recurrent bleeding is common and can be caused by the Helicobacter Pylori (H.Pylori) infection. Our aim was to evaluate the role of H.Pylori infection in UGI bleeding in PWH, using Serology and Stool antigen tests. Methods:Ninety patients with hereditary bleeding disorders, 30 patients(group A) with and 60 patients(group B) without a history of UGI bleeding, who were admitted to our Hemophilia Treatment Center, were included in the study. The prevalence of H.Pylori infection was investigated by stool antigen test, as well as serum serologic tests including IgG and anti-CagA. Results:Among 90 patients (81 men,16 women, mean age 31 ± 11 years), 66 patients with Hemophilia A,10 patients with Hemophilia B, 6 patients with VWD, and 3 patients with other factor deficiencies were evaluated for H.Pylori infection. About 46.7% of patients in group A, and 23.3% of patients in group B were anti-Cag A – positive in serum (P=0.02), while 76.7% of patients in group A and 51.7% of patients in group B had H.Pylori IgG antibodies in serum (P= 0.02). H.Pylori antigen in stool was positive in 76.7% in group A and 55% in group B (P=0.03). Gastro endoscopy was done to find the bleeding focus and further evaluations, in 19 patients with acute UGI bleeding. No statistically significant difference was found between type and severity of diseases and risk of UGI. Conclusion:There was a statistically significant differences in H.Pylori infection between patients with and without UGI bleeding history. So, H.Pylori infection should be considered as an important cause of UGI bleeding in PWH. We would recommend stool antigen test as a new and none-invasive screening test for diagnosis of H.Pylori infection in all patients with hereditary hemorrhagic disorders. Disclosures:Dolatkhah:Hematology and Oncology Research Center, Tabriz University of Medical Sciences: Employment, Research Funding. Khoshbaten:Hematology and Oncology Research Center, Tabriz University of Medical Sciences: Research Funding. Kermani:Hematology and Oncology Research Center, Tabriz University of Medical Sciences: Employment, Membership on an entity's Board of Directors or advisory committees, Research Funding. Sanaat:Hematology and Oncology Research Center, Tabriz University of Medical Sciences: Employment, Research Funding. Bonyadi:Hematology and Oncology Research Center, Tabriz University of Medical Sciences: Employment, Research Funding. Ghojazadeh:Hematology and Oncology Research Center, Tabriz University of Medical Sciences: Employment, Research Funding. Kermani:Hematology and Oncology Research Center, Tabriz University of Medical Sciences: Research Funding.

Contributions to the evolution of knowledge about hereditary hemorrhagic disorders.

Contributions to the evolution of knowledge about hereditary hemorrhagic disorders.

Genetic diagnosis of haemophilia and other inherited bleeding disorders

Inherited deficiencies of plasma proteins involved in blood coagulation generally lead to lifelong bleeding disorders, whose severity is inversely proportional to the degree of factor deficiency. Haemophilia A and B, inherited as X-linked recessive traits, are the most common hereditary hemorrhagic disorders caused by a deficiency or dysfunction of blood coagulation factor VIII (FVIII) and factor IX (FIX). Together with von Willebrand's disease, a defect of primary haemostasis, these X-linked disorders include 95% to 97% of all the inherited deficiencies of coagulation factors. The remaining defects, generally transmitted as autosomal recessive traits, are rare with prevalence of the presumably homozygous forms in the general population of 1:500,000 for FVII deficiency and 1 in 2 million for prothrombin (FII) and factor XIII (FXIII) deficiency. Molecular characterization, carrier detection and prenatal diagnosis remain the key steps for the prevention of the birth of children affected by coagulation disorders in developing countries, where patients with these deficiencies rarely live beyond childhood and where management is still largely inadequate. These characterizations are possible by direct or indirect genetic analysis of genes involved in these diseases, and the choice of the strategy depends on the effective available budget and facilities to achieve a large benefit. In countries with more advanced molecular facilities and higher budget resources, the most appropriate choice in general is a direct strategy for mutation detection. However, in countries with limited facilities and low budget resources, carrier detection and prenatal diagnosis are usually performed by linkage analysis with genetic markers. This article reviews the genetic diagnosis of haemophilia, genetics and inhibitor development, genetics of von Willebrand's disease and of rare bleeding disorders.

Carrier Detection and Prenatal Diagnosis of Hemophilia in Developing Countries

Hemophilia A and B, inherited as X-linked recessive traits, are the most common hereditary hemorrhagic disorders caused by a deficiency or dysfunction of coagulation factor VIII (FVIII) or FIX, respectively. Hemophilia is prevalent worldwide, without ethnic or geographic limitations, and remains a life-threatening and often disabling condition. Advances in molecular medicine in this century have markedly improved hemophilia treatment. However, management is still largely inadequate in developing countries. Therefore, carrier detection and prenatal diagnosis remain the key steps for the prevention of the birth of children with hemophilia in developing countries where patients with this coagulation disorder rarely live beyond childhood. Carrier detection and prenatal diagnosis are possible by direct or indirect genetic analysis of the F8 or F9 genes. In countries with more advanced molecular facilities and higher budget resources, the most appropriate choice in general is a direct strategy for mutation detection by prescreening techniques or direct mutation detection. However, in countries with limited facilities and low budget resources, carrier detection and prenatal diagnosis are usually performed by linkage analysis with genetic markers. This article suggests the possibilities of genetic diagnosis and a feasible strategy for carrier detection and prenatal diagnosis in families with hemophilia A and B in developing countries.

Spectrum of Inherited Bleeding Disorders in Indians

The incidence of hereditary hemorrhagic disorders may vary according to the country and ethnic origin. Von Willebrand disease has emerged as the most common hereditary hemorrhagic disease in the industrialized world. In this series of 966 patients diagnosed to have inherited bleeding disorders, hemophilia A was the most common and was seen in 410 (42.4%) of the patients followed by platelet function defects seen in 380 (39.4%) of the patients. It is thus concluded that, similar to the white population, hemophilia A remains the most common bleeding disorder in the Indian population, although this is closely followed by platelet function defects in India, which are quite rare in whites. Von Willebrand disease is relatively rare in the Indian population.

Candida in saliva of Brazilian hemophilic patients

Hemophilia is a common hereditary hemorrhagic disorder, however little is known about the oral microflora of hemophilic patients. The aim of this study was to quantify the Candida and identify its species in non-stimulated saliva of hemophilic patients, and consider its relationship with clinical factors influencing Candida carriage. This study comprised evaluation of 86 hemophilic patients of the Hematology Center/UNICAMP and 43 healthy subjects as controls. All patients were submitted to anamnesis, intraoral examination and unstimulated saliva collection. Candida counts and species identification were performed in salivary samples. Candida was present in 64% of the hemophilic patients and in 44% of the healthy controls. C. albicans represented 65% and 68% of the isolated species, in hemophiliacs and control group respectively, and C. tropicalis was the second most common species in both groups. These results indicate that hemophilic patients carry Candida more frequently and in higher counts than healthy controls, independently of oral clinical parameter considered, as viral infections, complete dentures, transfusions of hemoderivatives, and salivary flow.

Anestesia para septoplastia e turbinectomia em paciente portador de doença de von Willebrand: relato de caso

Although von Willebrands disease is the most common hereditary hemorrhagic disorder, there are few reports in Brazilian literature relating this disease to anesthesia. This report aimed at describing a case of general anesthesia for septoplasty and turbinectomy in a von Willebrands disease type I patient, prophylactically treated with desmopressin (1-deamine-8-D- arginine vasopressin, DDAVP) in the pre and postoperative period. A female patient, 19 years old, 58 kg, with hypothyroidism controlled with L-tiroxine (75 mg) had her von Willebrands disease manifested three years before after a wisdom tooth extraction with persistent bleeding in the postoperative period. To prevent new per and postoperative hemorrhagic episodes, patient was prophylactically treated with desmopressin (0.3 microg.kg-1). Anesthesia was induced with midazolam (2.5 mg), fentanyl (150 microg), droperidol (2.5 mg), lidocaine (60 mg), atracurium (30 mg) and metoprolol (4 mg), followed by tracheal intubation and ventilation under intermittent positive pressure. Anesthesia was maintained with 2% sevoflurane in a mixture of 50% oxygen and nitrous oxide. This technique provided a good heart rate and blood pressure control during surgery. Patient remained with a nasal tampon for 24 hours and no bleeding was observed at its removal. Patient was discharged the day after surgery uneventfully. There were no immediate or late postoperative bleeding. The prophylactic treatment with DDAVP associated to the anesthetic technique used in this case was effective in controlling peri and postoperative bleeding.

Helicobacter pylori infection in upper gastrointestinal bleeding in patients with hereditary hemorrhagic disorders

Background: In patients with hereditary bleeding disorders, upper gastrointestinal bleeding presents a life-threatening complication, while the role of Helicobacter pylori ( H. pylori) infection in this group of patients has not been fully clarified in the literature. The aim of the present study was to evaluate the role of H. pylori infection and of dental status in upper gastrointestinal bleeding in patients with hereditary hemorrhagic disorders (HHD). Methods: Thirty-seven patients with HHD (18 patients with and 19 without a history of upper gastrointestinal bleeding) and 26 control patients without HHD, who were admitted to our hospital for elective gastroscopy due to dyspeptic symptoms, were included in the study. Endoscopy was performed on all patients with gastrointestinal bleeding and on controls. ELISA was used to detect IgG, anti-CagA, and IgA antibodies to H. pylori in the serum and saliva of patients and controls. Moreover, dental status was examined using the decayed/missing/filled teeth index (DMFT) in all subjects. The χ 2-test was used for statistical analysis. Results: Some 64.8% of the patients and 65.4% of the controls had H. pylori IgG antibodies in serum ( P>0.1, NS) while 54.05% of the patients and 34.6% of controls were anti-CagA-positive in serum ( P=NS). However, 83 and 26.3% of the HHD patients with and without gastrointestinal bleeding, respectively, were serum anti-CagA-positive ( P<0.01) while 72 and 58%, respectively, were serum IgG-positive ( P=NS). H. pylori antibodies in saliva and the DMFT calculated index did not differ between the two subgroups. Subsequently, all serum anti-CagA-positive HHD patients received 1-week of triple H. pylori eradication therapy with omeprazole, clarithromycin, and amoxicillin orally. During a 2-year follow-up, none of these patients reported upper gastrointestinal bleeding. Conclusions: Although no statistically significant difference in H. pylori infection was found between HHD and controls, the CagA strain appeared more frequently in those HHD patients with a history of upper gastrointestinal bleeding. Given our results and the limited data available in the literature, we would recommend anti-CagA screening and therapy to all patients with HHD. However, further studies with a longer follow-up and a greater number of patients are necessary.

76-Year-Old Man With Retroperitoneal Bleeding After Prostatectomy

76-Year-Old Man With Retroperitoneal Bleeding After Prostatectomy

Retinal Hemorrhage Associated with Thrombasthenia

A healthy 20-year-old man presented with a spontaneous unilateral retinal hemorrhage. Because of a history of easy bruisability, we obtained hematologic studies and diagnosed thrombasthenia, a hereditary hemorrhagic disorder. The association of retinal hemorrhage and thrombasthenia is rare. Thrombasthenia and other platelet functional disorders are becoming better defined as tests for these abnormalities become more reliable and available. Patients with apparent spontaneous retinal hemorrhages of unknown origin should be questioned about hemorrhagic tendencies, and the various tests for hemorrhagic disorders should be obtained. The hematologic survey should include the more sophisticated tests of platelet function. Patients with thrombasthenia should avoid aspirin intake.

Blood Disorders in the Elderly.

I was somewhat surprised when I received this volume for review in that it had occurred to someone to write a hematology book whose scope was limited to the elderly. Pediatric hematology, fine; but geriatric hematology! It simply glosses over those blood diseases which are generally held to be peculiar to young children, and briefly covers the rest of hematology. Unfortunately, much of what seems rightfully to belong to the pediatrician cannot be ignored when dealing with older patients. All too often a hereditary hemolytic or hemorrhagic disorder first turns its hand to mischief in later years. Further, most of the remaining diseases may indeed be problems of young or middle aged adults. Despite my little quarrel with its skewed viewpoint, I do not think this is a bad book. The writing is direct and concise, and the format attractive and easy to read. References are not extensive but are