Immunological evaluation of beta-thalassemia major patients receiving oral iron chelator deferasirox

To determine the immune abnormalities and occurrence of infections in transfusion-dependent beta-thalassemia major patients receiving oral iron chelator deferasirox [DFX]. An observational study. Hematology Clinics, King Khalid University Hospital, Riyadh, Saudi Arabia, from July to December 2010. Seventeen patients with beta-thalassemia major [12 females, median age 26 years] receiving deferasirox [DFX] for a median duration of 27 months were observed for any infections and had their immune status determined. Immune parameters studied included serum immunoglobulins and IgG subclasses, serum complement [C3 and C4] and anti-nuclear antibody [ANA] level, total B and T-lymphocytes, CD4+ and CD8+ counts, CD4+/CD8+ ratio, and natural killer [NK] cells. Immunological parameters of the patients were compared with age, gender, serum ferritin level and splenectomy status. Lymphocyte subsets were also compared with age and gender matched normal controls. A considerable reduction in serum ferritin was achieved by DFX from a median level of 2528 to 1875 micromol/l. Serum IgG levels were increased in 7 patients. Low C4 levels were found in 9 patients. Total B and T-lymphocytes were increased in 14 patients each, while CD4+, CD8+ and NK cells were increased in 13, 12 and 11 patients respectively. Absolute counts for all lymphocyte subsets were significantly higher compared to the normal controls [p

Vanishing bile duct syndrome in a Hodgkin's lymphoma patient with fatal outcome despite lymphoma remission

Vanishing bile duct syndrome [VBDS] is a condition resulting from severe bile duct injury, progressive destruction, and disappearance of intrahepatic bile ducts [ductopenia] leading to cholestasis, biliary cirrhosis, and liver failure. VBDS can be associated with a variety of disorders, including Hodgkin's lymphoma [HL]. We describe a 33-year-old male patient who presented with lymphadenopathy and jaundice, and was diagnosed to have HL. Serum bilirubin worsened progressively despite chemotherapy, with a cholestatic pattern of liver enzymes. Diagnosis of VBDS was established on liver biopsy. Although remission from HL was achieved, the patient died of liver failure. Presence of jaundice in HL patients should raise the possibility of VBDS. This report discusses the difficulties of delivering chemotherapy in patients with liver dysfunction. HL-associated VBDS carries a high mortality but lymphoma remission can be achieved in some patients. Therefore, liver transplantation should be considered early in these patients.

Hypopigmentation of the skin due to imatinib mesylate in patients with chronic myeloid leukemia

Hypopigmentation is an infrequently reported adverse effect of imatinib mesylate [IM] in chronic myeloid leukemia [CML], but there are no reports from Arab or Saudi patients. Thus, we assessed the frequency and impact of hypopigmentation in patients with chronic myeloid leukemia [CML] taking IM in our institution in Riyadh. We studied 24 adult CML patients taking IM and followed from March to June 2008. Telephonic interviews with all the CML patients taking IM were conducted and case notes were reviewed. Findings were confirmed on a subsequent clinic visit by a physician. Demographic features, disease status, response to IM, presence and severity of skin changes and impact of these changes on the patients and the disease were noted. Eight [33%] patients [6 males, 2 females] developed hypopigmentation due to IM. All patients had newly diagnosed, chronic phase CML and received 400 mg IM daily. The median age of the affected group was 37 years [range 18-54 years]. Hypopigmentation developed during the first 3 months of treatment in 5 patients and 6 months or later in 3 patients. It was generalized in 7 patients and involved the hands and face in one patient. No photosensitivity was reported and none had other significant side effects. Hypopigmentation of the skin can develop in about one third of CML patients taking IM. Physicians taking care of CML patients should be aware of this and patients need to be warned before commencing IM, particularly in dark-skinned patients

Dyspnea, pulmonary function and exercise capacity in adult Saudi patients with sickle cell disease

To examine pulmonary function, dyspnea and exercise capacity in adult Saudi sickle cell disease SCD patients. The patients were recruited from the hematology clinic at King Khalid University Hospital in Riyadh from January to December 2005. The study involved 39 patients with stable SCD 20 women and 19 men, with a mean age of 22.7 +/- 7.1 years, hemoglobin level of 95.5 +/- 14.6 g/L and hemoglobin F level of 13.7 +/- 8.6%. Patients underwent pulmonary function tests PFT forced expiratory volume in first second [FEV1], forced vital capacity [FVC], and diffusion capacity of carbon monoxide [DLco] data are presented as a percentage of the normal prediction, a 6-minute walk test 6MWT and echocardiography. Dyspnea was assessed using the Borg score. The 6MWT data were compared to body mass index-matched healthy controls. Forty-one percent of SCD patients had mild dyspnea at rest, and this increased to 61% at the end of the 6MWT. Pulmonary function tests were abnormal in 51% [36% of patients had a restrictive pattern, 10% had isolated decrease in DLco, and 5% had a mixed restrictive-obstructive pattern]. The 6MWD was shorter in SCD patients compared to the controls [368 +/- 67 versus 407 +/- 47m, p = 0.005]. No hematological variables correlated with outcome variables. Chronic pulmonary complications in adult Saudi SCD patients are relatively mild but common. Pulmonary function in these patients differs from that published for African-origin SCD patients. This difference may reflect a different natural history of SCD in the 2 populations

Hypocalcemia due to hypoparathyroidism in Beta thalassemia major patients

This is a retrospective analysis of case records of b-thalassemia major patients who developed hypoparathyroidism [HPT]. The objective of this study was to assess the prevalence of hypocalcemia and hypoparathyroidism in b-thalassemia major patients being followed at King Khalid University Hospital [KKUH], Riyadh, Saudi Arabia. Patients and Diagnosis was based on low serum calcium [S/Ca], high serum phosphate [Po4], normal serum magnesium and alkaline phosphatase, and low serum parathyroid hormone levels. Other parameters analyzed included age, sex, serum ferritin levels, age of onset of HPT, any symptoms of hypocalcemia, and presence of other complications in these patients. Out of 40 patients, eight [20%] were diagnosed to have HPT. The mean age at diagnosis was 13.6 years [range 11-16 years], mean serum calcium was 1.88 mmol/L [range 1.58-2.04], mean serum ferritin was 7490 micro g/L [range 2000-23,064] and mean serum phosphate was 1.88 mmol/L [range 1.50-2.73]. Serum parathyroid hormone [PTH] levels were low in most of the patients. Only two patients [25%] had mild symptoms of hypocalcemia. Growth retardation was present in all patients, while four patients had liver dysfunction, two had diabetes mellitus and two had cardiac dysfunction. HPT due to iron overload may develop in a significant number of thalassemia major patients, especially when chelation therapy is not optimal, therefore, all thalassemics should be carefully watched for this complication from early in their second decade