Hypereosinophilic syndrome associated with polycythemia vera

A 60-year-old woman presented with relapse of polycythemia vera associated with hypereosinophilic syndrome (HES) with abnormal immunologic measures, including increased serum IgE and IgG levels, high levels of circulating immune complexes, rheumatoid factor, and antinuclear antibodies. Treatment with hydroxyurea was followed by a dramatic response of both the polycythemia vera and the HES, with return to normal of the abnormal immunologic measures. This case report documents that evidence of immunologic and myeloproliferative causes of HES may coexist in the same patient.     

Megakaryoblastic transformation of polycythemia vera associated with hypercalcemia

Leukemic transformation is not uncommon in polycythemia vera, particularly after treatment with chemotherapeutic agents. The leukemias that supervene are mostly of myeloid type but megakaryoblastic transformation is distinctly uncommon. We report a case of polycythemia vera terminating in megakaryoblastic leukemia with associated hypercalcemia.     

Myelodysplastic syndrome with trisomy 11 associated with polycythemia vera

A 52-year-old male with myelodysplastic syndrome (MDS) who had a prior history of polycythemia vera is reported. Chromosome analysis revealed that the bone marrow and blood cells at the MDS phase contained trisomy of chromosome 11 as the sole cytogenetic change. Trisomy 11 is rarely found in hematologic neoplasia, and all of the reported cases with trisomy 11 were diagnosed as having nonlymphocytic neoplasia. In this report, a correlation between the chromosome change and leukemia/MDS developed in polycythemia vera is discussed.     

Mirtazapine in progressive multifocal leukoencephalopathy associated with polycythemia vera

Progressive multifocal leukoencephalopathy (PML) is a usually fatal cerebral white matter disease found in patients with human immunodeficiency virus infection and other immunocompromised states. We present the case of a 63-year-old woman with polycythemia vera who developed a progressive focal neurological deficit with white matter abnormalities on magnetic resonance images of the brain that was proved on biopsy to be PML. She was treated with the serotonin reuptake inhibitor mirtazapine and remains neurologically stable, with resolution of cerebral lesions, >2 years after diagnosis. We propose that mirtazapine should be investigated further for use in PML.     

Screening for abdominal aortic aneurysms during cardiac catheterization

BACKGROUND: Many authorities advocate routine screening for abdominal aortic aneurysms (AAA). Patients undergoing cardiac catheterization often have risk factors for AAA. The purpose of this study was to evaluate the clinical utility of screening for AAA during cardiac catheterization. METHODS: We prospectively examined the abdominal aorta in 127 patients undergoing diagnostic cardiac catheterization. Panning down during left ventriculography was the primary method for imaging the abdominal aorta. However, if there was difficulty advancing the catheter into the abdominal aorta, a formal postero-anterior abdominal aortogram was obtained. Off-line analysis of aortic morphology and diameters was performed. RESULTS: We achieved adequate visualization of the abdominal aorta in 99 of 127 patients. Mean age was 67 years; 75% were male. Risk factors for AAA were common, and included: history of smoking (88%); angina (84%); hypertension (57%); hyperlipidemia (52%); previous myocardial infarction (39%); diabetes mellitus (30%); obesity (21%); congestive heart failure (17%); presence of peripheral vascular disease (14%); and previous peripheral vascular procedures (12%). Panning down during left ventriculography was performed in 89% of cases in the 30 degrees right anterior oblique position. Previously undiagnosed, asymptomatic abdominal aortic aneurysms were found in 8 of 99 patients (8%). The mean intraluminal aneurysmal diameter was 2.8 +/- 1.1 cm and the mean length was 5.4 +/- 1.8 cm. Abdominal ultrasonography was performed in 7 of these cases (1 patient refused), and the mean diameter measured was 3.8 +/- 1.4 cm. Peripheral vascular disease, previous peripheral vascular surgery and difficulty advancing the catheter during the procedure were associated by univariate analysis with the presence of AAA. Multivariate analysis demonstrated that difficulty threading the catheter was the only independent predictor of the presence of AAA (odds ratio = 11.1; 95% confidence interval = 4.6-26.6; p = 0.007). CONCLUSION: Undiagnosed abdominal aortic aneurysms can be identified during routine examination of the abdominal aorta during cardiac catheterization. Because screening for AAA during cardiac catheterization is inexpensive and easily performed, it should be a routine part of the examination.     

An unknown cause of aortic valve stenosis: polycythemia vera

Polycythemia vera (PV) is a chronic myeloproliferative disorder characterized by an abnormal increase in red blood cells. The involvement of the heart during the course of the illness represents a common cause of morbidity and it is linked to an increased thrombogenic risk subsequent to higher blood viscosity. In our study we evaluated by echocardiography a PV patient population. Our study enrolled 44 patients affected by PV. 17 of them were women and 27 were men. Mean patient age was 66.7. The average follow-up period was 5 years and the average duration of the illness was 5.7 years, since the time of diagnosis. All patients were evaluated quarterly by a cardiovascular objective examination and an ultrasound of the heart, with regard to platelet count and hematocrit (Ht) variations during the follow-up period, according to the therapy administered. Patients were treated with hydrossiurea and pipobroman and they underwent an eritrocitoapheresis in emergency conditions in which Ht levels rose too much, in spite of the myelosuppressive therapy. The echocardiographic assessment of the heart structure and function by the B mode technique revealed the presence of a sclerocalcific degeneration of the aortic valve in 58 % of patients, involving the aortic root more then the valve. An average trans-aortic flow velocity of 1.92 m/s was detected by Doppler technique; a stenosis was demonstrated in 11 patients (25.5 % of the entire population). After diagnosing the presence of a stenosis, we researched a possible cause of it. PV is a systemic disease well-known causing coronary thrombosis in a more or less high percentage of patients according to the record of cases taken into account. In our experience, more then thrombotic disease, found only in 13.4 % of patients, we detected a high prevalence and incidence of mild to severe aortic stenosis, found in 25.5 % of the sample studied. About all possible causes of stenosis, nowadays this results dependent of Ht values at moment of diagnosis, in the light of these results, it is reasonable to infer that aortic valve stenosis could depend by high haemodynamic stress on valve that is characteristic of polycythemic patients without chemotherapy.     

Complications associated with pediatric cardiac catheterization

Objectives. The aim of this study was to determine the relative risks of pediatric diagnostic, interventional and electrophysiologic catheterizations.Background. The role of the pediatric catheterization laboratory has evolved in the last decade as a therapeutic modality, although remaining an important tool for anatomic and hemodynamic diagnosis.Methods. A study of 4,952 consecutive pediatric catheterization procedures was undertaken.Results. Patient ages ranged from 1 day to 20 years (median 2.9 years). One or more complications occurred in 436 studies (8.8%) and were classified as major in 102 and minor in 458, with vascular complications (n = 189; 3.8% of procedures) the most common adverse event. Arrhythmic complications (n = 24) were the most common major complication. Death occurred in seven cases (0.14%) as a direct complication of the procedure and was more common in infants (n = 5). Independent risk factors for complications included a young patient age and undergoing an interventional procedure.Conclusions. Complications continue to be associated with pediatric cardiac catheterization. Efforts should be directed to improving equipment for flexibility and size, and finding alternative methods for vascular access. Patient age and interventional studies are risk factors for morbidity and mortality.     

以急性心肌梗死首诊的真红细胞增多症1例

急性心肌梗死发病率高,少部分急性心肌梗死继发于其他疾病,真性红细胞增多症的血栓发生机率高,但是以AMI首发并行介入治疗成功的个案少见。这些患者的病情复杂多变,治疗方法多在摸索与探讨。报道1例以PV合并AMI首诊的个案特点、诊断、治疗及随访情况,从实验室检查、药物选择、介入术式的选择几方面分析该单纯PV合并AMI,无其他危险因素的典型病例,以期探索规范化的诊疗及治疗的方案,减少死亡率,提高治愈率。     

Treatment of polycythemia vera with hydroxyurea

Conventional treatment of polycythemia vera (PV) with radioactive phosphorus or alkylating agents is associated with a significant excess of acute leukemia and cancer of the gastrointestinal tract and skin. There is thus a need for a nonmutagenic agent in the treatment of this disorder. Hydroxyurea (HU) was administered to 118 patients with a loading dose of 30 mg/kg/day for 1 week, which was then reduced to 15 mg/kg/day. Initial control of the elevated hematocrit and platelet count was achieved within 12 weeks in over 80% of patients. Long-term disease control was defined and the accumulative 1-year failure-free survival was 73% in the previously untreated patients and 59% in those patients previously treated with other myelosuppressive modalities. The HU was well tolerated and cytopenia, which generally occurred within the first 8 weeks of therapy, was transient and of little clinical significance. However, it is recommended because of this toxicity that HU be administered initially at a dose of 15-20 mg/kg/day. Three patients developed acute leukemia; two were untreated and one had had myelosuppressive therapy. Hydroxyurea is an effective agent in the treatment of PV, but continued assessment of its mutagenic potential is necessary.     

Therapy of polycythemia vera with myleran

Myleran was used in the therapy of nine relapses of polycythemia verain five patients. Clinical examinations, blood studies, and, in three instances,radioisotope tracer tests before and after treatment demonstrated the effectiveness, safety and simplicity of the treatment. Further trial of Mylerantherapy in polycythemia vera seems warranted.

Submitted on September 6, 1957 Accepted on December 16, 1957     

Subacute Budd-Chiari syndrome associated with polycythemia vera and factor V Leiden mutation

We describe a 48-year-old caucasian woman with a subacute Budd-Chiari syndrome attributed to the presence of polycythaemia vera, heterozygosity for the factor V Leiden mutation and the use of an oral contraceptive pill. Two diagnostic pitfalls were encountered. First, on CT scanning of the abdomen the still normally vascularized central part of the liver was initially judged as a hypervascular tumour. Second, there was difficulty to recognize the thrombocytosis/leukocytosis in relation with portal hypertension and hypersplenism. Our case illustrates the obstacles which can be faced during the diagnostical process in a subacute form of Budd-Chiari syndrome. Furthermore, our case illustrates the need for routinely screening of the factor V Leiden mutation in patients with Budd-Chiari syndrome.     

Carcinoid tumor of the duodenum and accessory papilla associated with polycythemia vera

Carcinoid tumors have been reported in a wide range of organs but most frequently involve the gastrointestinal tract; however, duodenal carcinoid tumors are rare. We report a 50-year-old male patient complaining of multiple melenas for 3 wk. The panendoscopy and endoscopic retrograde cholangiopancreaticography revealed swelling accessory papilla with an ulcer. The biopsy taken showed a carcinoid tumor. The lesion was removed by wide resection. Patient was found to have an abnormal blood cell count during the follow-up period with elevated levels of hemoglobin and hematocrit of 21.2 g/dL and 63.5%, respectively, thrombocytosis of 501 000/μL, and leukocytosis of 20 410/μL. He was diagnosed as a polycythemia vera by a hematologist after further evaluation. He received periodic phlebotomy and hydroxyurea treatment. The response was good and his hematocrit was stabilized by periodic phlebotomy in the range of 44-49% during the last 2 years. The possible origin of UGI bleeding by a duodenal carcinoid tumor, although rare, should be considered. There has been one case report of a duodenal carcinoid tumor that involved accessory papilla of the pancreas divisum and one case report of metastatic carcinoid tumor associated with polycythemia vera. It is different in our patient as compared with the latter report, which mentioned a polycythemia vera patient who was found to have a metastatic carcinoid in the 17 years follow-up period. Chemotherapy had been given before the carcinoid tumor was revealed. Our patient had no previous chemotherapy for polycythemia vera before he was found to have duodenal carcinoid tumor; this excludes the possibility of chemotherapy induced carcinoid tumor, although it had been suspected in the previous report. In our patient, the existence of both diseases may be by predisposition of each other since both diseases have an increased incidence of other neoplasm, or they may be coexistent incidentally.     

Acute leukemia following treatment of polycythemia vera and essential thrombocythemia with uracil mustard

Transformation to acute leukemia (AL) is known to occur in polycythemia vera (PV) and essential thrombocythemia (ET). Myelosuppressive therapy with agents such as 32P and alkylating agents increase this risk in both disorders. The alkylating agent, uracil mustard (UM), which is an effective agent for controlling thrombocytosis, has not been reported to be leukemogenic. We have treated 29 patients with UM (9 treated continuously and 20 treated intermittently): II with PV, 16 with ET, and 2 with myelofibrosis (MF). Three patients developed AL, two after continuous therapy. These two patients with PV had received the fourth highest and highest total dose of UM, and their duration of treatment was the third and fourth longest among the nine patients treated continuously, respectively. One out of 20 patients treated intermittently with UM developed AL. This patient (3) with ET had received the highest total dose of UM, and her duration of treatment was the longest among the 20 patients treated intermittently.