Thymoma with Concomitant Pure Red Cell Aplasia, Good's Syndrome and Myasthenia Gravis Responding to Rituximab.

Thymomas are often associated with a variety of autoimmune diseases, mostly myasthenia gravis. The association of thymomas with both pure red cell aplasia (PRCA) and Good's syndrome is exceedingly rare. To the best of our knowledge, the combination of a thymoma with manifestations of myasthenia gravis, PRCA, and Good's syndrome, as in our case herein, has not been described before in the medical literature. We present a 90-year-old man initially diagnosed with an asymptomatic thymoma. Later, he developed generalized muscle weakness and was found to have severe anemia. He was diagnosed with PRCA, myasthenia gravis and Good's syndrome. He responded to rituximab with restoration of bone marrow erythroid maturation and stabilization of red blood cell counts.

A nationwide non-interventional epidemiological data registry on myelodysplastic syndromes in Lebanon.

Myelodysplastic syndromes (MDS) are a heterogeneous group of clonal hematopoietic disorders characterized by peripheral blood cytopenias, blood cells dysplasia, and increased risk for progression to acute leukemia.Physicians should be vigilant in diagnosing MDS and should be aware of the contemporary therapies that are always in progress. Most of the data on MDS epidemiology and management comes from developed countries. The incidence and features of MDS in the Arab countries, among them Lebanon, are not known. We undertook a nationwide epidemiological registry study of all newly diagnosed MDS cases through 2010-2011. Patients were referred by 21 hematologists/oncologists practicing in 17 hospitals and medical centers distributed across the entire country. 58 patients (29 males and 29 females) with confirmed MDS were included. The calculated incidence rate of MDS was 0.71 per 100,000 people. The median age at diagnosis was 73 years (range 16-86). The most common complaints on presentation were fatigue (70.7%), weakness (60.3%) and pallor (43.1%). Most patients were diagnosed as refractory anemia with excess blasts (RAEB; 36.2%) and refractory cytopenia with multilineage dysplasia (RCMD; 32.8%). This paper constitutes the first epidemiological report on the incidence and specific subtypes of MDS in Lebanon.

Recurrent jejunal bleeding due to angiodysplasia in a Bernard-Soulier patient.

Angiodysplasia of the gastrointestinal tract consists of ectasia of the submucosal vessels of the bowels and may lead to acute or chronic bleeding. The presence of a coagulopathy will increase the frequency and the severity of gastrointestinal bleeding, whether spontaneously or medically. In literature, few cases of Bernard-Soulier syndrome associated with gastrointestinal angiodysplasia have been reported. We hereby present a female patient known to have Bernard-Soulier syndrome presenting with persistent bleeding due to jejunal angiodysplasia, which, to our knowledge, is the eighth reported case in the medical literature. The patient responded to tranexamic acid (antifibrinolytic agent) with gradual reduction in required transfusions until cessation of bleeding.

Chronic lymphocytic leukemia associated with myelodysplastic syndrome with ring sideroblasts.

Diagnosis of myelodysplastic syndrome/acute myeloid leukemia (MDS/AML) and chronic lymphocytic leukemia (CLL) in the same patient is extremely rare. We describe a 69-year-old CLL patient who developed MDS with ring sideroblasts 1 year after diagnosis of CLL and without any previous treatment. Diagnosis was based on flow cytometry, bone marrow aspirate morphology, and iron stain. Our findings indicate that the 2 disorders belong to 2 different hematopoietic clones in this patient. In cases of worsening anemia in CLL patients, it is recommended that an iron stain be performed on bone marrow aspirates to exclude a coexistent malignancy such as refractory anemia with ring sideroblasts.

Long-term outcome of adult acute lymphoblastic leukemia in Lebanon: a single institution experience from the American University of Beirut.

BACKGROUND AND OBJECTIVES: The most important studies about outcome of acute leukemia come from developed countries, whereas most of the patients with this disease are in developing countries. We report predictive and prognostic factors in patients with acute lymphoblastic leukemia (ALL) in a tertiary care center in a developing country. PATIENTS AND METHODS: We retrospectively reviewed the records of adult patients with acute leukemia who were referred to the American University of Beirut Medical Center between 1996 and early 2006. RESULTS: Of 105 patients, 36 (34%) patients were diagnosed with ALL, and included 19 (53%) males and 17 (47%) females with a median age of 34 years (range, 14-79 years). Induction chemotherapy with curative intent was administered to 34 (94%) patients. Twenty-seven patients received intrathecal chemotherapy as prophylaxis (n=24) or as treatment for CNS disease (n=3). Twenty-eight patients (82%) achieved complete remission (CR) after induction chemotherapy. The median overall survival (OS) time was 22 months and the five-year OS for ALL patients was 38%. The median disease-free survival (DFS) time was 12 months, while the five-year DFS was 38%. Multivariate analysis showed that age <40 years, WBC <30 X 109/L, achievement of CR after first induction, and CNS prophylaxis were predictive factors for OS and DFS. CONCLUSION: Despite limitations and the relatively low socioeconomic status of the Lebanese population, OS (38%) and DFS (38%) are quite similar to international data. Trends toward a higher CR and DFS in adults are due to intensified consolidation chemotherapy, the use of stem cell transplantation, and improvements in supportive care.

Plasmapheresis in asparaginase-induced hypertriglyceridemia.

BACKGROUND: Asparaginase is an essential component of the chemotherapy regimens during the induction and intensification phases for acute lymphoblastic leukemia and lymphoblastic lymphoma. The administration of asparaginase can cause elevation of the triglyceride levels. CASE REPORT: This study reports the case of an adult patient presenting with acute pancreatitis secondary to severe hypertriglyceridemia after asparaginase treatment during the consolidation phase of lymphoblastic lymphoma. This condition was successfully treated with plasmapheresis. CONCLUSIONS: Our case demonstrates that plasmapheresis in severe cases of hypertriglyceridemia offers a safe and efficient treatment option for symptomatic patients.

Duodenal mucosa-associated lymphoid tissue lymphoma successfully treated by radiation therapy.

Duodenal mucosa-associated lymphoid tissue (MALT) lymphoma is very rare, and little is known about its clinical characteristics, endoscopic and endosonographic features, and treatment. We hereby report a case of duodenal MALT lymphoma successfully treated by radiation therapy (RT). The patient was referred to us with epigastric pain and positive fecal occult blood testing. His symptoms failed to resolve with eradication therapy for a Helicobacter pylori infection that was diagnosed by a gastric biopsy performed elsewhere. Endoscopy at our institution revealed hypertrophy of the duodenal folds with erosions involving a third of the circumference few centimeters beyond the ampulla of Vater. Histopathologic and immunophenotypic features were consistent with a MALT lymphoma. There was no evidence of a H. pylori infection by gastric biopsy and urea breath test. Computed tomography scan of the abdomen and pelvis was normal. Endoscopic ultrasound showed thickening of the duodenal wall and hypoechoic infiltration into the submucosal layer. The patient was treated with RT with a complete response. Two and a half years later, he remains in complete clinical, endoscopic, and histopathologic remission. This case illustrates the importance of RT in patients with duodenal MALT lymphoma whose disease did not respond to H. pylori eradication.

Rituximab-induced acute thrombocytopenia: a report of two cases.

Rituximab use in B-cell malignancies has been widely favored by the acceptable toxicity profile of this drug. Episodes of rituximab-induced neutropenia have been reported in some patients, but severe acute thrombocytopenia is very unusual. Here, we report transient severe acute thrombocytopenia after rituximab infusion in two patients with, one hairy cell leukemia the other with mantle cell lymphomay. Interestingly, in both cases, thrombocytopenia was reversible in a few days without further therapeutic intervention. The mechanism of this side effect remains unclear. Previous reports suggested the presence of CD20 antigen on the platelets themselves or that soluble CD20 antigen in the circulation may cause an antigen-antibody reaction and immune-mediated cell lysis. It is noteworthy that the two cases reported here as well as the two previously published cases share massive bone marrow involvement by neoplastic B lymphocytes.

Four primary tumors of lung, bladder, prostate, and breast in a male patient.

We present a very rare case of quadruple cancers in a 65-year-old male patient. It is a case of both synchronous and metachronous primary malignant neoplasms occurring in four different organs. Immunohistochemical stains showed tumor cell nuclei to be negative for p53 over-expression. To our knowledge, this is the first documented case with this combination of primary tumors. The tumors included an adenosquamous cell carcinoma of the lung, transitional cell carcinoma of the urinary bladder, and adenocarcinomas of the prostate and the breast. We also review the medical literature for the possible causes of multiple primary malignant neoplasms.

Heterozygous prothrombin 20210G/A mutation, associated with hyperhomocysteinemia, and homozygous methylenetetrahydrofolate reductase 677C/T mutation, in a patient with portal and mesenteric venous thrombosis.

We herein report a 34-year-old man who was investigated for severe abdominal pain. Portal vein thrombosis (PVT) and mesenteric vein thrombosis (MVT) were diagnosed. An association with two predisposing factors for thrombosis was noted: (1) heterozygous factor II 20210G/A mutation and (2) homozygous methylenetetrahydrofolate reductase (MTHFR) 677C/T mutation with hyperhomocysteinemia. Our case is of particular interest because the patient reported herein, is homozygote for the MTHFR 677C/T mutation, while the only two other cases reported in the literature with similar gene mutations, were heterozygotes for the mutation.

Age distribution of breast cancer in Lebanon: increased percentages and age adjusted incidence rates of younger-aged groups at presentation.

BACKGROUND: Breast cancer is the most common cancer in Lebanese women. Lebanon has no national cancer registry and the American University of Beirut Medical Center (AUBMC) is one of the largest hospitals in Lebanon and has a fully operational cancer registry. Earlier studies showed that it sees about one third of all cancer cases in Lebanon. METHODS: All female breast cancer patients recorded at AUBMC between 1983 and 2000 were evaluated. We used the sex-specific age distribution of 1995 Lebanese Population and Housing Survey to estimate the age-specific incidence of breast cancer in Lebanon. The results were calculated as number and proportion of cases, 10-year age-specific incidence rates, crude rates and age standardized rates (ASR) per 100,000 population. The ASR per 100,000 population was estimated by the direct method with the use of the World Standard Population. RESULTS: Between 1983 and 2000, there were a total of 16421 cancers of which 8007 were in women. There were 2673 female breast cancers, averaging 148 cases per year (Range:94-202). Almost half of cases (49.1%) were in women below the age of fifty. The mean age was:49.8 years +/- 13.9 years. Ten-year age groups distribution showed that 4.7% were below 30 years of age, 16.1% were 30-39 years, 28.3% were 40-49 years, 26.3% were 50-59 years, 16.9 % were 60-69 years, 6.1% were 70-79 years and 1.6% were 80 years of age or older. Twenty-two patients (0.9 %) had their age missing in the records. Overall ASR was 30.6, for a crude rate of 27.7. Age adjusted incidence rate had its peak in women aged 50-59, followed by women 40-49 then 60-69 with values of 96.3, 79.9 and 77.4 per 100,000 respectively. We also noted 19 male breast cancer cases corresponding to 0.7% of the 2692 combined total. CONCLUSIONS: The percentage of women with breast cancer in Lebanon seen at AUBMC in pre-menopausal and younger-aged groups is higher than those reported from western countries. Our results emphasize the need to search for possible environmental, lifestyle and/or genetic risk factors in Lebanon. Our study also shows the importance of implementing early detection and screening programs which, along with high quality mammography and medical care, can have a positive impact on survival, especially in younger-aged women.