1/7. budd-chiari syndrome complicating restorative proctocolectomy for ulcerative colitis: report of a case.PURPOSE: This is a case of hepatic vein thrombosis presenting in a delayed fashion after proctocolectomy with ileal pouch-anal anastomosis for ulcerative colitis. Search for a causative thrombotic condition resulted in the diagnosis of polycythemia vera, a myeloproliferative disorder associated with hypercoagulability. The polycythemia was masked by an iron deficiency associated with the ulcerative colitis. methods: The history, physical, diagnostic modalities, and treatment for this patient are described, and the literature of budd-chiari syndrome associated with ulcerative colitis is reviewed. RESULTS: Six cases of budd-chiari syndrome in the setting of ulcerative colitis are reported in the literature from 1945 to 1997. CONCLUSIONS: Hepatic vein thrombosis is a rare complication of ulcerative colitis. The diagnosis of budd-chiari syndrome demands a thorough search for a hematologic condition predisposing to thrombosis. Our patient had a myeloproliferative disorder, polycythemia vera, that is associated with a hypercoagulable state. The disorder was masked by an iron deficiency associated with the ulcerative colitis. Recognition of the entity will permit successful treatment.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/7. An unusual cause of hypercalcemia in polycythemia vera: parathyroid adenoma.In this paper we describe a patient with polycythemia vera (PV), who presented with hypercalcemia due to a parathyroid adenoma. In November 1999, the patient was admitted to our hospital with meteorism and constipation. Her physical examination revealed plethora and hepatosplenomegaly. Laboratory data revealed hyperparathyroidism in addition to PV: Rbc 8 x 10(6)/mm3, Hct 63.7%, serum calcium 13.4 mg/dl, serum phosphorus 1.2 mg/dl, albumin 4.25 mg/dl, and alkaline phophatase activity 433 U/l. Intact parathyroid hormone level (iPTH) was 376 pg/ml (n.v.12-72 pg/ml). Twenty-four hour urinary calcium excretion was higher than normal (900 mg). A parathyroid adenoma was detected with Tc-99m sesta-MIBI scanning under the left lobe of the thyroid gland and an ultrasonographic examination of the neck also supported the diagnosis. The patient was recommended for surgery. The histopathological examination confirmed the diagnosis. Postoperatively, iPTH dropped to 53.4 pg/ml at the 15 th minute and to 33.5 pg/ml at the first hour. The calcium level was 7.5 mg/dl one hour after the operation. Five days later, Hct was 40.8%. This case represents a rare association between PV and primary hyperparathyroidism, and may provide evidence for a causal link between PTH and polycythemia vera in our patient. In conclusion, this case indicates that the differential diagnosis of hypercalcemia and polycythemia vera should also include the possibility of a parathyroid tumor in addition to malignancy.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
3/7. A case of transition of polycythemia vera to chronic neutrophilic leukemia.Chronic Neutrophilic leukemia (CNL) is a rare myeloproliferative disorder characterized by a persistent increase of mature peripheral neutrophils, myeloid hyperplasia in bone marrow, hepatosplenomegaly, elevated neutrophil alkaline phosphatase (NAP) and absence of philadelphia chromosome, with no evidence of infection or malignancy sufficient to mimic a leukemoid reaction. CNL has been associated with multiple myelomas in many reported cases, but transition of polycythemia vera (PV) to CNL is very rare. An 81-year-old female patient, who had undergone intermittent phlebotomy following the diagnosis of PV 8 years previously, was admitted to our hospital due to lower back pain. A physical examination showed a splenomegaly 2 cm below the costal margin, with tenderness of the thoracic and lumbar spine area. A peripheral blood examination showed a WBC count of 91,800/microL (neutrophil 88%) with a rare immature form, hemoglobin of 9.1 g/dL and a platelet count of 1,661,000/microL. Her NAP score was 58. The bone marrow examination showed 95% cellularity, with an M:E ratio of 10:1, increased megakaryocytes with normal morphology and the absence of myelofibrosis. Chromosomal studies showed no philadelphia chromosome. A radiological examination showed compression fractures of the vertebrae and spinal cord compression. No underlying disease causing a leukemoid reaction was detected. With iron replacement, the hemoglobin level failed to increase over 12 g/dL. Therefore, it was concluded to be a transition of PV to CNL. After administration of hydroxyurea and vertebroplasty, the symptom improved and the WBC count was sustained below 40,000/microL.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
4/7. Recovery of splenic infarction with anti-platelet treatments and platelet-apheresis in polycythemia vera.Thrombo-embolic events in coronary and peripheral arteries, and cerebral, pulmonary, portal, hepatic, and deep veins are seen in 27-45% of patients with polycythemia vera (PV). A 79-year-old man was admitted with complaints of pruritus increasing after bath and left upper abdominal pain radiating to left shoulder for two months. On physical examination, ruddy and hyperemic appearances of his face and conjunctiva, tenderness on the left upper quadrant, and splenomegaly were noted. Hemoglobin level was 16.6g/dl, hematocrit 53.8%, white blood cell count 26x10(9)/l, and platelet count 1.032x10(9)/l. Bone marrow aspiration and biopsy revealed hypercellularity, megakaryocytic hyperplasia and dysplasia. The leukocyte alkaline phosphatase score was 190. The levels of serum vitamin B12 and D-dimer were 316 pg/ml and 744 ng/ml, respectively. Arterial O2 saturation was 96%. Red cell mass was measured as 43 ml/kg using radionuclide 51Cr labelled erythrocyte scintigraphy. On cytogenetic analysis, deletion of 20q was found. Computed tomography of whole abdomen showed diffuse splenomegaly and two hypodense areas indicating splenic infarction in 2.5x2 and 3.5x3 cm diameters in subcapsular localization of the spleen. The patient was treated with therapeutic platelet-apheresis, 40 mg/day aspirin, analgesic drugs, and 3g/day hydroxyurea. After 1.5 months, platelet counts dropped to less than 500x10(9)/l and splenic infarcts were not detected on computed tomography. splenic infarction may be the first evidence of thrombosis in PV. The reduction of platelet counts with platelet-apheresis, anti-platelet drugs, and careful clinical observation may be satisfactory in the treatment of splenic infarction.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
5/7. Primary polycythaemia in a dog.A 7-year-old, crossbred, desexed bitch had a fluctuating illness of more than 6 months duration, with signs of inappetence, reduced exercise tolerance, restlessness, panting, black diarrhoea, and red eyes and ears. Haematological examination disclosed persistent polycythaemia, reticulocytosis, normoblastaemia , and normal total plasma protein concentration when not dehydrated. splenomegaly was present, but there was no evidence of significant pulmonary, cardiac or renal disease on physical, radiographic or electrocardiographic examination. Further investigations revealed normal arterial Po2 and oxygen saturation, and increased total erythrocyte volume. Primary polycythaemia was diagnosed. The dog was rendered anaemic by bleeding, then treated with a single injection of radioactive phosphorus. The patient has had no recurrence of signs and remains well 50 months later.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
6/7. Spontaneous remission of polycythemia vera: clinical and cell culture characteristics.A 20-year-old woman presented with polycythemia vera and was treated with phlebotomy alone for eleven years, following which all clinical manifestations of the disease disappeared. The clinical remission with normal physical findings and normal peripheral blood counts has persisted for a further 11 years. Erythroid colony culture results have paralleled the clinical state. Initial bone marrow cultures revealed spontaneous growth of erythroid burst-forming units (BFU-E). Subsequent cultures of peripheral blood cells throughout most of the period of spontaneous clinical remission have revealed little or no spontaneous growth of BFU-E. This suggests a suppression of the abnormal stem cell clone during the period of remission.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
7/7. Leukemic transformation in three patients with polycythemia vera. Analysis of the clinicopathological features and N-ras gene mutation.Thirty-three patients were diagnosed as having polycythemia vera (PV) from 1973 to 1993 in our institution. Of these patients, three who had been treated with alkylating agents, progressed to acute non-lymphocytic leukemia (ANLL). Their physical findings and the laboratory data were similar to those patients who did not become leukemic. To investigate the association with leukemic evolution, we examined N-ras oncogene activation in those patients who progressed to acute leukemia. Point mutations in codons 12 and 13 were not always detected, suggesting that the N-ras gene did not influence occurrence of ANLL in our patients.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |