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Idiopathic cyclic thrombocytopenia.

Cyclic thrombocytopenia (CTP) is an uncommon disorder characterized by periodic fluctuations in platelet counts, typically resulting in episodes of thrombocytopenia alternating with normal platelet counts. While some CTP cases are associated with a primary hematologic disease, most are idiopathic. Patients with CTP are frequently misdiagnosed as idiopathic thrombocytopenic purpura (ITP) because CTP has clinical features very similar to ITP. When evaluating patients with suspected ITP, CTP should always be included in the differential diagnoses because CTP generally does not respond to standard ITP treatments, including corticosteroids, splenectomy, and intravenous immunoglobulin. Two clinical features relatively unique to CTP besides periodic thrombocytopenia are rebound thrombocytosis unrelated to recent splenectomy and platelet nadirs occurring during menses. When a diagnosis of CTP is made, patients must be offered a period of observation, as many may not require treatment. If treatment is clinically indicated, the literature suggests that hormonal therapy provides the best response.

Humans↗

Occurrence of a variant Philadelphia translocation, t(10;22), in de novo acute megakaryoblastic leukemia.

Acute megakaryoblastic leukemia (AMegL) in adults is a very rare subtype of acute myeloid leukemia (AML) and is characterized by a larger diversity of chromosomal abnormalities than the other subtypes, including 3q21q26 changes, aberrations of chromosomes 5 and 7, and the t(9;22)(q34;q11). We report the case of a 24-year-old patient with de novo AMegL and thrombocythemic cell count. Diagnosis was established with a bone marrow biopsy, and cytogenetics with G-banding revealed a t(10;22), which by FISH, was found to be a variant Philadelphia translocation involving chromosome 10q in all 20 metaphases analyzed. We believe that this is the first report of de novo AMegL with this chromosomal abnormality, and its possible correlation with morphology and thrombocytosis is discussed.

Adult↗

Translocation (3;8)(q26;q24): a recurrent chromosomal abnormality in myelodysplastic syndrome and acute myeloid leukemia.

We identified a reciprocal translocation between chromosomes 3 and 8, with breakpoints at bands 3q26 and 8q24, in five patients with myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML). The t(3;8)(q26;q24) was the sole cytogenetic aberration in two patients, was associated with trisomy 13 in one patient, and occurred with monosomy 7 in two patients. In three patients, the AML or MDS developed 36, 52, and 57 months following chemotherapy for soft tissue sarcoma, mantle cell lymphoma, and diffuse large B-cell lymphoma, respectively; in these three patients, the neoplasms were considered to be therapy-related. All five patients displayed marked trilineage dysplasia and variable degrees of cytopenias, with marked thrombocytosis noted in one patient and a normal platelet count in another patient. All patients were treated with combination chemotherapy; at writing, four were still alive and one had died during a follow-up period ranging from 1 to 16 months. We conclude that the t(3;8)(q26;q24) is a recurrent translocation associated with therapy-related MDS/AML or de novo AML, and is frequently associated with monosomy 7.

Acute Disease↗

Sepsis and elevated liver enzymes in a patient with inflammatory bowel disease: think of portal vein thrombosis.

A 42-year old man, 1 year previously diagnosed with ulcerative colitis after an emergency subtotal colectomy with formation of an ileostomy because of severe colitis with perforation, was admitted with sepsis and jaundice. The liver enzymes were elevated and blood cultures were positive for Streptococcus milleri. Magnetic resonance imaging showed a complete thrombosis of the main stem of the portal vein with occlusion of the left branch. Intravenous antibiotic therapy combined with heparinisation led to complete recanalisation of the thrombus. Portal vein thrombosis is a rare complication of inflammatory bowel disease and has been described in only 10 patients thus far. Multiple aetiologic factors may be responsible in relation to inflammatory bowel disease, such as hypercoagulability, thrombocytosis and abdominal sepsis. In patients with inflammatory bowel disease, unexplained sepsis and abnormal liver function tests, the possibility of an acute portal vein thrombosis should be considered and investigated, because unrecognised it may have serious long-term complications.

Adult↗

Hematoma of a congenitally bicuspid aortic valve in a patient with polycythemia vera and the antiphospholipid antibody syndrome.

We present a novel case of a hematoma involving a congenitally bicuspid aortic valve. The patient was a 47-year-old woman with polycythemia vera and antiphospholipid antibody syndrome. She was treated with anagrelide for thrombocytosis. The patient developed a valvular hematoma which caused clinically significant aortic stenosis and regurgitation. Consequently, the patient underwent surgery with insertion of a porcine bioprosthesis. Eighteen months postoperatively the patient developed a large prosthetic thrombus with severe aortic stenosis requiring reoperation.

Antiphospholipid Syndrome↗

Increased plasma levels of stromal-derived factor-1 (SDF-1/CXCL12) enhance human thrombopoiesis and mobilize human colony-forming cells (CFC) in NOD/SCID mice.

OBJECTIVE: Stromal-derived factor-1 (SDF-1/CXCL12) is chemotactic for lympho/hematopoietic stem cells. We have previously shown that increasing peripheral blood (PB) levels of SDF-1 with adenovectors expressing human SDF-1 complementary DNA (ad-SDF-1) leads to hematopoietic stem cell mobilization as well as migration of megakaryocytes and thrombocytosis in mice. Herein, we studied the in vivo effects of ad-SDF-1 and of an analogue peptide of SDF-1 (CTCE-0214) on human hematopoiesis in a xenotransplant model. MATERIALS AND METHODS: Sublethally irradiated (300 cGY) NOD/SCID mice transplanted with human cord blood mononuclear cells (CB MNC) were injected with ad-SDF-1 (10(9) plaque forming units, i.v., x 1) or CTCE-0214 (10 mg/kg/dose, i.v. q 24 hours x 7). Effects on megakaryocytopoiesis (CD41+ cells and platelets) as well as stem cell mobilization were monitored. RESULTS: CB MNC in NOD/SCID mice are able to differentiate into CD41+ cells and platelets, peaking at week 9 at a mean of 3.7 x 10(3)/microL. i.v. injection of ad-SDF-1 increased human CD41+ cells by day 4 in PB and was followed by an increase in human platelet production by day 5, with return to baseline by day 30. Human colony-forming cells (CFC) were mobilized from bone marrow to spleen (by day 6-13) and to PB (by day 13). Human CD34+ and CD33+ cells were mobilized by this treatment as well. A novel SDF-1 peptide agonist (CTCE-0214) also mobilized human CFC and enhanced human thrombopoiesis. CONCLUSION: SDF-1 and its analogue may be of clinical value in stimulating platelet recovery after chemo/radiation treatment as well as in stem cell mobilization.

Adenoviridae↗

Fever of unknown origin: temporal arteritis presenting with persistent cough and elevated serum ferritin levels.

BACKGROUND: Fever of unknown origin (FUO) at the present time is most frequently caused by neoplasm and less commonly by infection. Currently, collagen vascular diseases (CVDs) are an uncommon cause of FUO because most are readily diagnosable by serologic methods and do not remain undiagnosed for sufficient time to present as FUOs. CVDs presenting as FUOs not readily diagnosable with specific tests include late-onset rheumatoid arthritis, adult juvenile rheumatoid arthritis, and polymyalgia rheumatica/temporal arteritis (TA). TA, or giant cell arteritis, is an uncommon arteritis of the mid- and large-sized extracranial arteries of the head and neck and is a rare cause of FUO. TA is characterized by headache, scalp tenderness, jaw pain on chewing, and sudden loss of vision. Fever, anorexia, weight loss, and night sweats may also be present. With TA, respiratory symptoms occur in 9% and are the presenting feature in 4%. Laboratory abnormalities associated with TA include a highly elevated erythrocyte sedimentation rate, anemia, and thrombocytosis, and mildly increased alkaline phosphatase/serum transaminases. PATIENT: We present a patient with FUO caused by TA whose predominant presenting symptom was persistent cough that overshadowed head and neck symptoms of TA. To the best of our knowledge, this is the first case of TA presenting as an FUO, with a highly elevated serum ferritin level. RESULTS: We conclude that highly elevated serum ferritin levels in patients with FUO should alert the clinician to consider TA in the differential diagnosis.

Aged↗

Portal vein thrombosis after splenectomy in pediatric hematologic disease: risk factors, clinical features, and outcome.

BACKGROUND/PURPOSE: A retrospective study was performed to evaluate risk factors, clinical features, and treatment modalities of portal vein thrombosis (PVT) after splenectomy in pediatric hematologic disease. METHODS: Sixty-eight patients who underwent splenectomy for various hematologic diseases were evaluated with regard to age, sex, blood count, and splenic mass. Patients who developed PVT were also reviewed for clinical features, treatment modalities, and outcome. RESULTS: Patients with PVT (n = 4, 5.88%) and without PVT (n = 64, 94.2%) had a mean age and female/male ratio of 13.2 years (range, 10-16 years) and 4:0, and 10.2 years (range, 1-16 years) and 29:35, respectively. Postoperative thrombocyte levels and splenic mass with and without PVT was 804 x 10(3)/mm(3) and 752.5 g, and 465.2 x 10(3)/mm(3) and 441g, respectively. Three patients with PVT presented with abdominal pain, fever, and vomiting. The diagnosis of PVT was made by Doppler ultrasonography in all patients including the asymptomatic case. Protein C, protein S, and antithrombin III levels were mostly decreased and/or normal and di-dimer levels were increased and/or normal after the development of PVT. Antiplatelet (acetylsalicylic acid) and antithrombotic therapy (low molecular weight heparin) were treatment agents. None of the patients needed surgery. During a mean follow-up period of 55.5 months, by Doppler ultrasonography, 1 patient was found to be free of thrombosis, whereas 1 had partial thrombosis. Two patients developed cavernomatous transformation leading to portal hypertension. CONCLUSIONS: Portal vein thrombosis is a rare but significant complication of splenectomy done for hematologic diseases. According to our results, female gender and decreased levels of coagulation inhibitors seem to be risk factors in addition to previously mentioned thrombocytosis and greater splenic mass. Doppler ultrasonography may be performed in all patients after splenectomy to screen PVT. In the presence of well-known risk factors, prophylactic antiplatelet and antithrombotic therapy should be considered after splenectomy.

Adolescent↗

CD34/CD117 positivity in assessment of prognosis in children with myelodysplastic syndrome.

Myelodysplastic syndromes (MDS) are a heterogeneous group of clonal stem cell disorders that are characterized by morphology identifying dysplastic changes in one or more cell lineages, peripheral blood cytopenias and a propensity to evolve into secondary acute myeloid leukemia (AML). CD34 is commonly expressed in all types of childhood leukemias, whereas CD117 is a reliable and specific marker to detect leukemia cells committed to myeloid lineage. Co-expression of CD34/CD117 may strongly suggest the diagnosis of AML (Rytting ME. Pediatric myelodysplastic syndromes. Curr hematol Rep 2004;3(3):173-7. May; Uçkan D, Hiçsönmez G, Yetgin S, Gürgey A, Cetin M, Karaağaoğlu E, et al. CD34/CD117 co-expression in childhood acute leukemia. Leukemia Res 2000;24:201-6.). We describe the case of a 22 month-old-girl with MDS and Down syndrome who was presented with severe anemia and thrombocytosis at diagnosis, transformed into AML-M7. In our patient, CD34 and CD117 markers were positive on the blast cells of the BM 6 months before the chemotherapy decision. As the disease progressed, CD34/117 co-existence was increased and MDS transformed into AML. As a result, an increase in CD34 and CD117 positivity of the BM blast cells may be associated with a higher risk of leukemic transformation.

Antigens, CD34↗

Predictors of wound complications after laryngectomy: A study of over 2000 patients.

OBJECTIVES: To identify risk factors for and the rate of wound complications after laryngectomy in a large, prospectively collected national dataset, and to generate a predictive model. Study design We used the National Surgical Quality Improvement Program (NSQIP) registry created by the Department of Veterans Affairs (VA) to identify patients undergoing total laryngectomy from 1989 to 1999 (n = 2063). We linked these data to inpatient and outpatient VA administrative records to capture data for prior radiation. Over 20 preoperative and intraoperative risk factors were analyzed using bivariate techniques. Those significant at the P < 0.01 level were analyzed with logistic regression and conjunctive consolidation to identify independent predictors of wound complications. RESULTS: The overall wound complication rate was 10.0%. In adjusted analyses, prolonged operative time (>10 hours, odds ratio = 2.10, 95% confidence interval: 1.32-3.36), exposure to prior radiation therapy (OR =1.63, 1.07-2.46), presence of diabetes (OR =1.78, 1.04-3.04), preoperative hypoalbuminemia (OR =1.90, 1.32-2.74), anemia (OR =1.59, 1.07-2.36), and thrombocytosis (OR =1.48, 1.04-2.10) were independently associated with postoperative wound complications. A prognostic model using three variables-prior radiation therapy, diabetes, and hypoalbuminemia-provided excellent risk stratification into three tiers (6.3%, 13.7%, 21.7%). CONCLUSIONS: Preoperative radiation, prolonged operative time, low albumin, and diabetes were independently associated with postoperative wound infections. These results will help to identify patients at risk for wound complications, thus allowing for heightened surveillance and preventive measures where possible.

Diabetes Complications↗

Adult Kawasaki disease: report of two cases and literature review.

OBJECTIVES: To describe 2 cases of adult Kawasaki Disease (KD) and to review the medical literature to better define the epidemiological, clinical, laboratory, histopathological, cardiovascular, and therapeutic aspects of adult KD compared with pediatric KD. METHODS: Report of 2 cases, and review of the literature using a Medline search from 1967 to June 2003. RESULTS: Including our 2 cases, there are 57 reports of adult KD, 74% among patients aged 18 to 30 years. Nine cases of KD associated with human immunodeficiency virus (HIV) infection were described, suggesting that an immunocompromised state may predispose to this syndrome. The incidence of specific diagnostic criteria was roughly similar in adults and in children. However, cheilitis, meningitis, and thrombocytosis were observed in a larger percentage of children, while arthralgia, adenopathy, and liver function abnormality were more common in adults. Although adult KD often was diagnosed after the acute phase, when a significant beneficial effect from gammaglobulin infusion could not be expected, this treatment did appear to shorten the course of the disease. Coronary aneurysms were less frequent in adults than in children. Prognosis was more favorable in adults, with less cardiovascular complications and no deaths. CONCLUSIONS: Adult KD is a rare condition, which may go unrecognized. Other known disease processes with similar clinical presentations such as hypersensitivity drug reaction and toxic shock syndrome must be ruled out. For adult KD, exclusion criteria such as absence of hypotension, visceral impairment, staphylococcal infection, and any drug able to induce a drug hypersensitivity reaction are suggestive of the diagnosis, in the presence of the inclusion criteria, rash, conjunctival effusion, oropharynx changes, extremity changes, or adenopathy.

Adult↗

Catastrophic Kawasaki disease or juvenile Polyarteritis nodosa?

OBJECTIVE: Juvenile Polyarteritis nodosa (PAN) and Kawasaki Disease (KD) are disseminated vasculitides of unknown cause affecting small- and medium-sized vessels in children. We present an unusually severe case that fulfilled criteria for both KD and PAN. The diagnosis, overlapping clinical features, and treatment options for the 2 diseases are discussed. METHODS: A 3-year-old girl with systemic vasculitis is presented. We compare our case to 4 other cases reported in the literature which presented with a similar diagnostic dilemma. A review of the medical literature and a qualitative analysis of the diseases were performed, with emphasis on overlapping features, atypical cases, and treatment options. RESULTS: Many features of KD and PAN are shared; however, there are some clinical features that could help differentiate one from the other. Fever, weight loss, rash, abdominal pain, arthritis, coronary arteritis, peripheral gangrene, anemia, leukocytosis, thrombocytosis, and elevated C-reactive protein are among many of the features that are shared by both diseases. However, KD also has unique clinical features that include conjunctivitis, changes in the lips and mouth, desquamation of the fingertips, and gallbladder hydrops, whereas renal involvement in KD is rare. CONCLUSIONS: Occasionally juvenile PAN and KD share clinical manifestations, and when they do, it may be impossible to differentiate between them. Treatment should be directed according to the severity and persistence of these clinical manifestations.

Child, Preschool↗

Platelet counts are persistently increased following simultaneous pancreas and kidney transplantation.

BACKGROUND: Increased platelet counts has been reported to be a sequela of pancreas transplantation and even incriminated in the increased rate of thrombosis of pancreas grafts. The aim of the study was to measure the platelet counts after simultaneous kidney-pancreas transplantations compared to kidney transplants alone in diabetic patients. METHODS: This retrospective case-control study included 57 patients who received simultaneous pancreas and kidney transplants (SPK), from 1985 to 2000 and had functioning grafts for more than 1 month. The control patients were 38 type I diabetic recipients of kidney transplants alone (KTA), matched for sex, era, and immunosuppression. The platelet counts, white cell counts, and hemoglobin were analyzed on the preoperative day, weeks 1 to 6, 3 months, 6 months and 1 year. RESULTS: The mean age of the SPK group was significantly lower than that of the KTA group (39.8+/-8.3 versus 48.2+/-11.7, P<.01). Significantly higher platelet counts were demonstrated during weeks 2 to 6, which persisted at 3 months and at 1 year among the SPK compared to the KTA group. Although significantly higher white cell counts and lower hemoglobin levels were seen among the SPK versus KTA group during weeks 3 to 6, it did not persist after 3 months. CONCLUSION: The mean platelet counts of patients with simultaneous pancreas and kidney transplantation was significantly higher than that of diabetic patients with kidney transplants alone. This thrombocytosis persisted up to the first year and cannot be explained by an increased amount of blood loss or higher infectious complications in the SPK group. Routine antiplatelet prophylaxis is recommended in this group of patients.

Case-Control Studies↗

The impact of age in the outcome of patients with advanced or recurrent cervical cancer after platinum-based chemotherapy.

OBJECTIVES: Cervical cancer is a disease of middle-aged and elderly but still there are young women diagnosed with advanced disease that is incurable with local treatment and is treated with platinum-based combination chemotherapy. It is unknown whether these young patients have a poorer outcome compared to older patients or whether elderly patients have inferior outcome than younger patients when treated with combination chemotherapy. METHODS: We compared the outcome between young (<35), elderly (>70) and middle-aged (35-70) women who were treated with platinum-based combination chemotherapy for advanced, recurrent or persistent disease. RESULTS: Two hundred and eighteen patients were included in our database. The baseline clinical and disease characteristics were not different between age groups but anemia and thrombocytosis were more frequent in younger patients. Median survival for all patients was 13.4 (95%CI 11-15.8) months while survival of patients<35 years of age was 9 months (95% CI 5.8-12), of patients older than 70 was 10 months (95% CI 6.9-13) of patients 35 to 70 years of age was 14.5 months (95% CI 11-18) (p=0.004). Multiple factors were significant for survival in univariate analysis but only weight loss, pain score and relapse inside an irradiated filed were significant predictors of outcome in multivariate analysis. CONCLUSIONS: Very young (<35) and elderly (>70) patients have a worse prognosis after treatment with combination chemotherapy for advanced or recurrent cervical cancer. Nevertheless, this difference is not significant when adjusted for other prognostic factors.

Adult↗

Radionuclide therapy of hematologic disorders.

32P is effective therapy for polycythemia and primary thrombocytosis. The Polycythemia Vera Study Group is comparing radioactive phosphorus with alkylating agents to determine relative efficacy. Less well investigated is the effectiveness of 32P vs. busulfan in chronic granulocytic leukemia. Endolymphatic administration of radiopharmaceuticals may play a role in the therapy of infradiaphragmatic lymphoma. Among the radionuclides that have at times been used in hematology are 32P, 198Au 24Na, 76As, 89Sr, 52Mb, 54Mn, 91Y, 95Zr, 95Cb, 111Ag, 109Pd, 131I, 185W, and 192Ir. As stated, 32P has proven single most efficacious agent. The hematologic diseases that have been treated include both malignant and benign conditions. Among the malignant conditions are polycythemia vera, agnogenic myeloid metaplasia, thrombocythemia, leukemia, Hodgkin's disease, and multiple myeloma. Hemophilia, and Osler--Weber--Rendu disease are among the benign entities in which the agents have been tried. Polycythemia and thrombocythemia remain those in which the greatest success has been achieved.

Gold Radioisotopes↗

Significance and correlations of molecular analysis results in patients with Philadelphia chromosome-negative chronic myelogenous leukemia and chronic myelomonocytic leukemia.

PURPOSE: Several investigators have documented a rearrangement of the breakpoint cluster region (bcr) in selected patients with a morphologic diagnosis of chronic myelogenous leukemia (CML) but no abnormality of the Philadelphia chromosome (Ph) by cytogenetic studies. Our intention was to systematically investigate the incidence of the bcr rearrangement in such patients, and to correlate the findings with patient characteristics, response to therapy (especially alpha interferon treatment), and overall prognosis. PATIENTS AND METHODS: Molecular analysis studies were performed in 40 patients with Ph-negative CML (23 patients) and myelomonocytic leukemia (CMML; 17 patients). RESULTS: Rearrangement of the breakpoint cluster region (bcr) was detected in 11 of the 23 patients with Ph-negative CML (48 percent), indicating the presence of the abnormal molecular events in Ph-positive CML without documentation of the Ph cytogenetic abnormality. None of the 17 patients with CMML had the bcr rearrangement. Patients with Ph-negative CML and the bcr rearrangement had characteristics similar to those of patients with Ph-positive disease. These included a younger age, higher white blood cell counts, a higher incidence of thrombocytosis and basophilia, and a lower occurrence of thrombocytopenia. The leukocyte alkaline phosphatase score was not a helpful distinguishing feature. Among 21 patients receiving alpha interferon-based regimens, response to therapy was significantly better among patients with Ph-negative disease and the bcr rearrangement (seven of seven, 100 percent), compared with those without the bcr rearrangement (one of six, 17 percent), or patients with CMML (two of eight, 25 percent) (p less than 0.01). At this time of follow-up, only one of the 11 patients with Ph-negative CML and the bcr rearrangement had died from complications of allogeneic bone marrow transplantation, compared with three deaths among the 12 patients with Ph-negative CML and no bcr rearrangement, and 11 deaths among the 19 patients with CMML. CONCLUSION: We conclude that molecular studies help in better understanding the nosology of Ph-negative CML, and define a subgroup of patients with clinical, therapeutic, and prognostic correlations similar to those of patients with Ph-positive CML.

Adolescent↗

Inapparent polycythemia vera: an unrecognized diagnosis.

PURPOSE: The Polycythemia Vera Study Group (PVSG) has established useful criteria for the diagnosis of polycythemia vera. In some circumstances, an increase of plasma volume (PV) masks that of red cell mass (RCM), with hemoglobin (Hb) and hematocrit (Ht) remaining normal. This defines the concept of inapparent polycythemia. PATIENTS AND METHODS: One hundred and three patients seen in the hematology unit with the diagnosis of polycythemia vera were studied. There were 55 males and 48 females with a median age of 59 years. Ninety-five patients fulfilled the PVSG criteria. Spontaneous erythroid colonies and low serum erythropoietin level confirmed the diagnosis in the 8 other cases. Patients were classified according to Hb and Ht level. RESULTS: Group A consisted of 85 patients with increased Hb and Ht defined, respectively, by Hb > 18 g/dl, Ht > 0.52 in males and Hb > 16 g/dL, Ht>0.47 in females. Group B included 18 patients (17%) with inapparent polycythemia vera (IPV) defined by a normal Hb and Ht value at diagnosis. In this group, the reasons to perform RCM were as follows: splenomegaly associated with increased platelets and/or leucocytes counts (n = 8), portal vein thrombosis (n = 5), increased platelets or leucocytes counts without splenomegaly (n = 3), and isolated splenomegaly (n = 2). The two groups were balanced in terms of age, sex, leucocyte, serum iron, and platelet level. Hemoglobin and Ht levels were significantly different between the two groups. The difference between the PV was indeed highly significant. The mean PV increase was + 9.5% (nL < +20%) in group A versus + 36.3% in group B (P < 0.00005). Red cell mass was not different between the two groups. CONCLUSIONS: Increased Hb or Ht should constitute the sole criteria for RCM determination. In the context of portal vein thrombosis, isolated hyperleucocytosis, thrombocytosis, or splenomegaly, a RCM should be performed. The frequency of IPV remains to be specified but the diagnosis of polycythemia vera is probably underestimated.

Adult↗

Peripheral retinal nonperfusion associated with chronic myeloid leukemia.

PURPOSE: To report a case of peripheral retinal nonperfusion and chronic myeloid leukemia in a 23-year-old woman. DESIGN: Observational case report. METHODS: A complete ophthalmic and systemic evaluation was performed. RESULTS: Ophthalmic examination revealed peripheral retinal nonperfusion with retinal neovascularization in both eyes. Fluorescein angiography of both eyes showed a marked midperipheral and peripheral avascular retina temporally with arteriovenous anastomosis and seafan neovascularizations. Blood work showed no abnormalities, although marked leucocytosis (up to 750 x 10(9)/l) and thrombocytosis (646 x 10(9)/l) were present in 1998 when the patient was diagnosed with leukemia. Following treatment, the patient has been in remission. CONCLUSIONS: Peripheral retinal nonperfusion with retinal neovascularization may occur as a complication of chronic myeloid leukemia. In contrast to other studies describing this association, our patient had a bilateral peripheral retinal nonperfusion with seafan neovascularizations without relapse of the myeloid leukemia and without any of the other retinal signs associated with chronic myeloid leukemia, such as tortuosity of veins, intraretinal or preretinal hemorrhages, and cotton-wool exudates.

Adult↗