An Overview and Update of Chronic Myeloid Leukemia for Primary Care Physicians.

Chronic myeloid leukemia (CML) accounts for approximately 15% of adult leukemias. Forty percent of patients with CML are asymptomatic, in whom the disease is detected solely based on laboratory abnormalities. Since the introduction of tyrosine kinase inhibitor therapy in 2001, CML has become a chronic disease for the majority of patients. Primary care physicians may be the first to recognize a new diagnosis of CML. In patients with known CML, the primary care physician may be the first to detect disease progression or adverse effects to therapy. This article provides an overview of the clinical presentation, diagnostic approach, and treatment considerations of CML.

Management of immune-mediated cytopenias in pregnancy.

Immune-mediated cytopenias are a well-described complication of pregnancy. Appropriate recognition and treatment are important in order to limit maternal and fetal morbidity and mortality. First line treatment options are fairly well-established for these entities. Refractory disease may be difficult to manage because treatment choices are limited by known or unestablished risk to the fetus. While the use of new agents, such as romiplostim and rituximab, has been reported, their safety in pregnancy is not known. This article summarizes immune cytopenias seen in pregnant patients, and it also discusses management of these cytopenias, and provides practical strategies for the treatment of these challenging conditions.

Response to imatinib therapy in adult patients with chronic myeloid leukemia in Saudi population: a single-center study.

No study has been published yet in the Arab world regarding response and outcome of imatinib in patients with chronic myeloid leukemia (CML). This study evaluated a total of 122 patients with CML treated with imatinib between 2001 and 2012. Survival, hematologic, cytogenetic and molecular responses and adverse events were assessed. The 5-year overall survival (OS), event free survival (EFS) and progression-free survival (PFS) rates were: 95.4 ± 2.3%, 81.4 ± 4.6% and 90.8 ± 3.2%, respectively. Significant differences in OS (p = 0.001), EFS (p = 0.001) and PFS (p = 0.001) were noted when patients were stratified by cytogenetic response. Survival by Sokal risk groups was not significant (p = 0.293). Complete hematologic response was achieved in 94 patients (93.1%), cytogenetic response in 84 (83.2%), major molecular response in 62 (61.4%) and complete molecular response in 34 (33.7%). This article presents the first evidence on the effectiveness of imatinib in patients with CML from Saudi Arabia and highlights similarities and differences in response patterns in published studies.

Management of immune cytopenias in patients with systemic lupus erythematosus - Old and new.

There are various immune cytopenias associated with systemic lupus erythematosus (SLE). The most common one is anemia; however, there are different etiologies for the anemia caused by SLE. Anemia could be due to chronic disease, secondary to renal insufficiency, blood loss, drug induced or autoimmune hemolysis. There are other very rare causes of anemia secondary to SLE which include red cell aplasia, aplastic anemia, and microangiopathic hemolytic anemia. Treatment of the anemia would be according to the cause. Leukopenia, neutropenia, and lymphopenia are hematologic complications associated with SLE, and in majority of cases no treatment is required. Thrombocytopenia is one of the complications of SLE and is usually treated by steroids. However, there are significant numbers of patients which will either not respond to or relapse after treatment. This article summarizes immune cytopenias seen in patients with SLE, and it also discusses management of these cytopenias.

Revisiting use of growth factors in myelodysplastic syndromes.

Myelodysplastic syndromes (MDS) represent a heterogeneous group of clonal hematologic neoplasms characterized by morphologic dysplasia, aberrant hematopoiesis and peripheral blood refractory cytopenias. MDS is recognized to be associated with an increased risk of symptomatic anemia, infectious complications and bleeding diathesis, as well as a risk of progression to acute myeloid leukemia, particularly in patients with a high IPSS score. The advent of use of hematopoietic growth factors such as granulocyte colony-stimulating factor (G-CSF) and recombinant erythropoietin (EPO) has improved symptoms in MDS patients in addition to some data that suggest there might be an improvement in survival. G-CSF is an effective therapeutic option in MDS patients, and it should be considered for the management of refractory symptomatic cytopenias. G-CSF and EPO in combination can improve outcomes in appropriate MDS patients such as those with lower-risk MDS and refractory anemia with ring sideroblasts (RARS) . This article reviews use of growth factors for lower-risk MDS patients, and examines the data for G-CSF, EPO and thrombopietic growth factors (TPO) that are available or being developed as therapeutic modalities for this challenging disease.

Management of haematological malignancy in pregnancy.

The incidence of haematological malignancies in pregnancy ranges from 1 in 1000 to 1 in 10,000. International prospective epidemiology, management and outcome data are important to gain further knowledge of haematological malignancies in pregnancy. The management of haematological malignancies in pregnancy is complex and requires a multidisciplinary approach. The clinician and mother need to address both maternal and fetal well-being. The mother should be provided with the necessary information and support to make informed decisions regarding the pregnancy and disease management. The haematological malignancies are a diverse group with varied presenting features, pathophysiology, treatment options, levels of urgency to commence treatment in pregnancy, effect on maternal and fetal outcome and overall prognosis. We have reviewed the published research in this area, and provide concise up-to-date guidance on the management of haematological malignancies in pregnancy.

Menorrhagia and bleeding disorders.

PURPOSE OF REVIEW: Menorrhagia affects 30% of women in reproductive age. Once referred to a gynaecologist, possible causative bleeding disorders are not routinely investigated and the risk of surgical intervention is high. This may lead to an increase in surgical complications and a negative health and psychological impact on women as well as an unnecessary financial burden on the health service. RECENT FINDINGS: Although the estimated community prevalence of bleeding disorders is 2%, these disorders are consistently reported to affect 10-20% of women with objectively documented menorrhagia and to be even higher in adolescents. Recently, underlying bleeding disorders, particularly von Willebrand's disease and platelet function disorders, have been found to be prevalent in women with menorrhagia. This article critically appraises the current literature in this field. SUMMARY: In the UK, 20% of all women, and 30% in the USA, have a hysterectomy before the age of 60; menorrhagia is the main presenting problem in at least 50-70%. In approximately 50% of cases, no organic pathology is determined, and dysfunctional uterine bleeding is diagnosed. Diagnosis and management of bleeding disorders may possibly reduce the need for surgical intervention, leading to a positive impact on women and the health service.

Serum erythropoietin values in erythrocytoses and in primary thrombocythaemia.

Serum erythropoietin (Epo) values were estimated in samples from 125 patients with erythrocytosis to examine the specificity and sensitivity of reduced and raised values in the diagnosis of polycythaemia vera (PV) and secondary erythrocytosis (SE) respectively. Additionally, Epo values were estimated in samples from 49 patients with primary thrombocythaemia (PT) to determine whether Epo values were altered. We found high specificity (92%) and moderate sensitivity (64%) of low serum Epo values (below the reference range) in the diagnosis of PV, and also poor sensitivity (47%) of raised Epo values in the diagnosis of SE. Raised Epo values were not observed in PV patients with Hb > 14.0 g/dl and were only observed in one PV patient with a relatively low Hb recovering from a gastro-intestinal haemorrhage. Raised Epo values occurred in some patients with apparent erythrocytosis (AE) and idiopathic erythrocytosis (IE), mainly at normal (rather than raised) Hb values (< 16 g/dl). Low Epo values occurred in a few AE, IE and SE patients at higher Hb values (> 16 g/dl). Low Epo values were less specific for PV when the Hb was raised, while raised Epo values were less specific for SE when the Hb was not raised. Approximately one third of patients with PT had a low (below the reference range) Epo value, this being associated with a high normal Hb (> 14 g/dl, P < 0.001) and showing a trend towards association with absence of treatment. The high normal Hb values were in turn associated with an increased incidence of thrombotic events (P < 0.05). These findings could influence the future investigation and management of PT patients.