Myomatous erythrocytosis syndrome:A case report

BACKGROUND Uterine myoma is the most common benign tumor among women and is often accompanied by anemia.Here,we report the case of a patient with a very large leiomyoma but with a hemoglobin level as high as 197 g/L.After undergoing hysterectomy,all her hematological parameters returned to normal.Immunohistochemical staining of her myoma for erythropoietin showed strong positivity,which suggested that erythropoietin may be the cause of her erythrocytosis.A multidisciplinary team played a significant role in treating the disease.CASE SUMMARY A 47-year-old woman visited our department complaining that her abdomen had been continuously growing for the past 2 years.After careful examinations,she was suspected of having a very large leiomyoma.She was also diagnosed with erythrocytosis because her RBC count was 6.49×10^(12)/L,hemoglobin was 197 g/L.Following a multidisciplinary team consultation,bilateral ureteral stents were placed,and 800 m L blood was removed by phlebotomy.The patient then underwent hysterectomy and bilateral salpingectomy.She recovered well from the operation,and her hemoglobin level decreased sharply following the surgery.Low-molecular-weight heparin was administered daily to prevent postoperative thrombosis.She was discharged from the hospital on the fourth postoperative day.Two months later,all her hematological parameters returned to normal.Pathological analysis of the myoma revealed that it was a benign leiomyoma,with partial hyalinization,and strong positivity for erythropoietin in immunohistochemical staining suggested that erythropoietin may be responsible for the erythrocytosis.CONCLUSION Erythropoietin ectopically produced from the myoma was responsible for the erythrocytosis in this patient.A multidisciplinary team is strongly recommended.

Erythrocytosis caused by giant chromophobe renal cell carcinoma:a case report indicating a 9.year misdiagnosis of polycythemia vera

Background:Erythrocytosis,a rare paraneoplastic syndrome,generally occurs in patients with clear cell renal cell carcinoma and has never been reported in patients with chromophobe renal cell carcinoma.Case presentation:We report a case of a young man suffering from a giant(22-cm) mass on his left kidney.Because of a history of polycythemia vera,the patient had been treated for the condition for 9 years.Radical nephrectomy was successfully performed,and the postoperative pathologic examination confirmed a diagnosis of chromophobe renal cell carcinoma.Unexpectedly,the symptom of erythrocytosis disappeared after the surgery.Further examination and analysis were performed,and we finally attributed his erythrocytosis to chromophobe renal cell carcinoma.Conclusions:Chromophobe renal cell carcinoma could cause erythrocytosis,but the clear-cut mechanism needs further research.Secondary erythrocytosis such as those related with renal tumors should be taken into consideration during the diagnosis of polycythemia vera.

Post-transplant erythrocytosis after kidney transplantation:A review

Post-transplant erythrocytosis(PTE)is defined as persistently elevated hemoglobin>17 g/dL or hematocrit levels>51%following kidney transplantation,independent of duration.It is a relatively common complication within 8 months to 24 months post-transplantation,occurring in 8%-15%of kidney transplant recipients.Established PTE risk factors include male gender,normal hemoglobin/hematocrit pre-transplant(suggestive of robust native kidney erythropoietin production),renal artery stenosis,patients with a well-functioning graft,and dialysis before transplantation.Many factors play a role in the development of PTE,however,underlying endogenous erythropoietin secretion pre-and post-transplant is significant.Other contributory factors include the renin-angiotensin-aldosterone system,insulin-like growth factors,endogenous androgens,and local renal hypoxia.Most patients with PTE experience mild symptoms like malaise,headache,fatigue,and dizziness.While prior investigations showed an increased risk of thromboembolic events,more recent evidence tells a different story-that PTE perhaps has lessened risk of thromboembolic events or negative graft outcomes than previously thought.In the evaluation of PTE,it is important to exclude other causes of erythrocytosis including malignancy before treatment.Angiotensin converting enzyme inhibitors(ACE-I)and angiotensin receptor blockers(ARBs)are the mainstays of treatment.Increased ACE-I/ARB use has likely contributed to the falling incidence of erythrocytosis.In this review article,we summarize the current literature in the field of post-transplant erythrocytosis after kidney transplantation.

Pheochromocytoma as a cause of repeated acute myocardial infarctions,heart failure,and transient erythrocytosis:A case report and review of the literature

BACKGROUND Pheochromocytoma is a rare catecholamines-secreting tumor arising from chromaffin cells in the adrenal medulla.It classically presents with paroxysmal hypertension,headaches,palpitations,sweating,and metabolic disorders.Atypical presentations such as acute myocardial infarction,heart failure,cardiomyopathy,stroke,and transient erythrocytosis have been infrequently documented.CASE SUMMARY We describe the case of a 72-year-old man diagnosed with pheochromocytoma presenting with non-ST segment elevation myocardial infarction,heart failure,and transient erythrocytosis with nonobstructed coronary arteries.This was his second heart attack.The patient was previously diagnosed with myocardial infarction,and an immense mass was found on the left adrenal gland 3 years prior.Based on clinical and laboratory findings,a diagnosis of pheochromocytoma was confirmed.His coronary angiogram showed nonobstructed coronary arteries except for a myocardial bridge in the left anterior descending branch.This was a form of type-2 myocardial infarction.The myocardial cell lesions were caused by sudden secretion of catecholamines by the pheochromocytoma.Even more atypically,his hemoglobin level was obviously elevated at admission,but after a few days of treatment with an alpha-adrenergic receptor blocker,it dropped to normal levels without additional treatment.CONCLUSION Pheochromocytoma may be a cause of acute myocardial infarction,heart failure,and transient erythrocytosis.

Relative Monocytosis in Patients with Erythrocytosis

Polycythemia vera (PV) is characterized by leukocytosis, thrombocytosis, and low mean capsular volume (MCV). No specific hematological findings are observed in patients with erythrocytosis of other origins. However, recombinant erythropoietin (EPO) increases the number of mononuclear cells in the peripheral blood. Therefore, we hematologically examined monocytes in patients with erythrocytosis. The medical records of 48 erythrocytosis patients (hemoglobin: men, >18.5 g/dL;women, >16.5 g/dL) collected between August 2005 and July 2012 were reviewed for age, gender, hematology, and serum erythropoietin level. Patients were divided into 3 groups: PV (N = 17), reactive erythrocytosis (RE, N = 25), and EPO-elevated erythrocytosis (EPO-E, N = 6). We compared monocytes in the hemogram among the 3 groups and examined the correlation between hematological data and serum EPO level. The percent monocytes in the hemogram were 3.4% ± 0.3%, 7.4% ± 0.4%, and 12.3% ± 1.1% for the PV, RE, and EPO-E groups, respectively, which were significant (p < 0.05). Absolute monocyte counts in the EPO-E group (1.0 ± 0.1 × 109/L) were significantly higher than those in the PV (0.6 ± 0.1 × 109/L) and RE (0.5 ± 0.3 × 109/L) groups. There were 2 (12%), 0, and 3 (50%) patients with monocytosis (>1.0 ′ 109/L) in the PV, RE, and EPO-E groups, respectively. A significant correlation was observed between percent monocyte or absolute monocyte count and serum EPO level. In conclusion, monocyte count in hemograms may be useful for diagnosing EPO-elevated erythrocytosis.

Alterations of red blood cell immunoadherence function in hepatitis B patients

AIMS To investigate the alterations of RBC immunoadherence function in patients with various hepatitis B. METHODS RBCC3bRR,RBCICRR and serum CIC levels were measured in 42 patients with acute and chronic hepatitis B at ac- tive and convalescence stages. RESULTS RBCC3bRRs at the active/acute stage of various hepatitis were decreased.They were 13,54%±5,23% in AH, 7.61%±4.12% in AFH,and 16.18%±6.10% in CH, respectively,all of which were lower than those in normal persons (18.12%±3.91% ).At the quiescent/recovery stage of various hepatitis,the RBCC3bRRs were increased significantly.The changes of RBCICRR and serum CIC level were contrary to those of RBCC3bRR. CONCLUSIONS RBC immunoadherence function is decreased in acute and chronic hepatitis.The decrease is in direct proportion to the severity of the diseases.

Laparoscopic hysterectomy as optimal approach for 5400 grams uterus with associated polycythemia: A case report

BACKGROUND Uterine myomas are the most common benign gynecological soft tissue tumors in women but polycythemia associated with these tumors is rare.Polycythemia is significantly associated with risk of venous thromboembolism(VTE),which is further increased in case of a large pelvic mass and obesity.Here we report the surgical challenges in the case of an obese patient with a giant fibromatous uterus and associated polycythemia.CASE SUMMARY A 42-year-old obese woman was referred to our department for treatment for a huge fibromatous uterus.She suffered of pelvic pressure and abdominal distension and reported severe dyspnea from several months.Laboratory analyses including hemoglobin(Hb)19.2 g/dL and hematocrit(Hct)59.7%were indicative of polycythemia.Arterial blood gas analysis showed arterial oxygen pressure(pO2)of 81.5 mmHg.Abdominal computed tomography confirmed a giant fibromatous uterus measuring 28.2 cm×17 cm×25 cm.To reduce the thromboembolic risk,the patient underwent low molecular weight heparin,phlebotomy twice before surgery,and we opted for a laparoscopic hysterectomy.The uterus weighed 5400 g and the histology confirmed a diagnosis of leiomyoma.We did not find increased erythropoietin levels in the supernatant of the myomatous uterine tissue.There were no complications.On postoperative day 1,the Hb and the Hct levels normalized to 13.3 g/dL and 41.7%,respectively.Arterial blood gas analysis after surgery showed a pO2 of 144.7 mmHg.These results suggested the diagnosis of myomatous erythrocytosis syndrome.The patient was discharged on the second postoperative day in very good condition with no symptoms.CONCLUSION We believe this is the first reported laparoscopic hysterectomy in a patient with a giant uterus,polycythemia and obesity.Multiple VTE risk factors warranted a laparoscopic approach.

Anesthetic management of a child with double outlet right ventricle and severe polycythemia: A case report

BACKGROUND Double outlet right ventricle(DORV)is a rare and complex congenital heart defect,and the surgical repairs vary with type and pathophysiology consequences.Due to prolonged progressive hypoxemia,severe polycythemia is common in patients with DORV,which ultimately leads to coagulation dysfunction and increases the risk of thrombosis and infarction.Consequently,the anesthetic management is challenging and how to manage severe polycythemia and avoid hypoxia-related complications in such patients is of great significance.CASE SUMMARY Herein,we report the anesthetic management of a 10-year-old female patient with a DORV.She lived in the low-oxygen Qinghai-Tibet Plateau,and presented with severe polycythemia(hemoglobin,24.8 g/dL;hematocrit,75%).She underwent a modified Fontan surgery,which was satisfactory and without any perioperative complications.Our anesthetic management highlights the importance of perioperative hemodilution in decreasing the risk of thromboembolism and the importance of correcting coagulopathy in preventing hemorrhage.CONCLUSION Anesthetic management is challenging in rare cyanotic congenital heart disease patients with severe polycythemia.It is important to adopt perioperative hemodilution and correction of coagulopathy in preventing thrombosis and hemorrhage.