First report of pediatric hematopoietic stem cell transplantation activities in the eastern mediterranean region from 1984 to 2011: on behalf of the pediatric cancer working committee of the eastern mediterranean blood and marrow transplantation group.

To describe the hematopoietic stem cell transplantation (HSCT) activities for children in the Eastern Mediterranean (EM) region, data on transplants performed for children less than 18 years of age between 1984 and 2011 in eight EM countries (Egypt, Iran, Jordan, Lebanon, Oman, Pakistan, Saudi Arabia and Tunisia) were collected. A total of 5187 transplants were performed, of which 4513 (87%) were allogeneic and 674 (13%) were autologous. Overall, the indications for transplantation were malignant diseases in 1736 (38.5%) and non-malignant in 2777 (61.5%) patients. A myeloablative conditioning regimen was used in 88% of the allografts. Bone marrow (BM) was the most frequent source of stem cells (56.2%), although an increasing use of PBSC was observed in the last decade. The stem cell source of autologous HSCT has shifted over time from BM to PBSC, and 80.9% of autologous HSCTs were from PBSCs. The donors for allogeneic transplants were matched-related in 94.5% of the cases, and unrelated transplants, mainly cord blood (99%) in 239 (5.5%) cases. This is the first report to describe the pediatric HSCT activities in EM countries. Non-malignant disorders are the main indication for allogeneic transplantation. Frequency of alternate donor transplantation is low.

Juvenile idiopathic oligoarthritis: analysis of 42 cases in Jordan.

We conducted a retrospective chart review for all 42 patients, 9 males and 33 females, diagnosed with juvenile idiopathic oligoarthritis between July 1995 and October 2004 at the paediatric rheumatology clinic in King Hussein Medical Centre. Age range was 1.2-15.2 years, mean 3.6 (standard deviation 2.4) years. Of those <6 years, 80% were females. Most patients (64%) had monoarthritis; the knee was the most common joint affected (71%). Two patients had developed extended oligoarthritis. Nine (21%) had asymptomatic uveitis: 6 females (P = 0.350), 7 (77%) <3 years of age (P = 0.301) and 7 (77%) with antinuclear antibodies (ANAs) (P = 0.071). Eighteen (43%) females were positive for ANAs. Most patients had undergone unnecessary, lengthy work-up and treatment before diagnosis.

Juvenile idiopathic oligoarthritis: analysis of 42 cases in Jordan

We conducted a retrospective chart review for all 42 patients, 9 males and 33 females, diagnosed with juvenile idiopathic oligoarthritis between July 1995 and October 2004 at the paediatric rheumatology clinic in King Hussein Medical Centre. Age range was 1.2- 15.2 years, mean 3.6 [standard deviation 2.4] years. Of those < 6 years, 80% were females. Most patients [64%] had monoarthritis; the knee was the most common joint affected [71%]. Two patients had developed extended oligoarthritis Nine [21%] had asymptomatic uveitis: 6 females [P = 0.350], 7 [77%] < 3 years of age [P= 0.301].and 7 [77%] with antinuclear antibodies [ANAs] [P = 0.071]. Eighteen [43%] females were positive for ANAs. Most patients had undergone unnecessary, lengthy work- up and treatment before diagnosis

Familial Mediterranean fever in children: a single centre experience in Jordan.

Familial Mediterranean fever is quite prevalent among Arabs. We reviewed the files of 56 patients diagnosed with familial Mediterranean fever and followed up at King Hussein Medical Centre in Jordan over 4 years for their clinical profile, course, genotype, treatment and complications. There were 30 males and 26 females with a mean age at onset of 5.2 years. Abdominal pain (79%) was the commonest manifestation, followed by arthritis (13%) and chest pain (4%). Family history was positive in 50% of patients. Regarding treatment, 97% of patients responded well to colchicine, and amyloidosis was not documented in any patients after 5 years follow-up. The commonest genotype was M694 (64%), followed by heterozygous M694V-V726A (23%) and E148Q (8%).

Familial Mediterranean fever in children: a single centre experience in Jordan

Familial Mediterranean fever is quite prevalent among Arabs. We reviewed the files of 56 patients diagnosed with familial Mediterranean fever and followed up at King Hussein Medical Centre in Jordan over 4 years for their clinical profile, course, genotype, treatment and complications. There were 30 males and 26 females with a mean age at onset of 5.2 years. Abdominal pain [79%] was the commonest manifestation, followed by arthritis [13%] and chest pain [4%]. Family history was positive in 50% of patients. Regarding treatment, 97% of patients responded well to colchicine, and amyloidosis was not documented in any patients after 5 years follow- up. The commonest genotype was M694 [64%], followed by heterozygous M694V- V726A [23%] and E148Q [8%]

Successful Bone Marrow Transplantation in a Child with X-Linked Hyper-IgM Syndrome.

A 13-year-old boy was diagnosed at the age of three years as having hyper IgM Immunodeficiency syndrome (HIgM). It is a rare congenital disease characterized by recurrent infections and very low level of serum immunoglobulin (IgG, IgA) and elevated IgM. Conservative treatment with antibiotics and regular intravenous immunoglobulin (IVIG) was not satisfactory. At the age of five, the patient developed Hodgkin's lymphoma, which was treated successfully with chemotherapy. Experience with Bone Marrow Transplantation (BMT) in such cases is limited as a definitive treatment for this kind of syndromes. He was transplanted from his HLA-matched sister, and three years post BMT follow-up he showed good clinical recovery and immunoreconstitution.

Comparison of Flow Panel Reactive Assay (PRA) TM Specific Test with Complement Dependent Cytotoxicity (CDC) to Define the HLA Antibodies Specificity: A Preliminary Study.

To determine the specificity of flow cytometry based assay for HLA antibody screening prior to transplantation, we evaluated 11 positive sera that were tested by both Flow Panel Reactive Assay (PRA) TM class I and class II specific beads after initial FlowPRA TM . HLA specificity was compared with previous data from the complement dependent cytotoxicity (CDC) testing of these samples. We found that five samples (22%) were more HLA class II specific (DR- or DQ-) by FlowPRA TM than CDC. One sample, negative by FlowPRA TM screening, was shown to be HLA class I and class II reactive by FlowPRA TM -specific test. Class I specificity was only defined by CDC in this sample. Two other samples, shown to be HLA class I and class II reactive by FlowPRA TM screening and FlowPRA TM -specific test, were found to be HLA class I reactive only in the first sample and HLA class II reactive only in the second sample by CDC. Our study suggests that FlowPRA TM -specific test has higher specificity and sensitivity than CDC in identification of HLA class II specificity than CDC. Nevertheless, FlowPRA TM -specific test failed to identify precisely the HLA specificity in samples with broad-spectrum specificity, such as those that have HLA specific antibodies directed against large number of shared epitopes. A software protocol for specificity analysis might help overcome this problem. Also, studies involving larger number of samples are required to validate our findings.

Adverse reactions of intravenous immunoglobulin.

OBJECTIVE: To study the frequency, type, and severity of adverse reactions to intravenous immunoglobulin infusions and possible preventable measures. METHODS: We prospectively studied the frequency, type, and severity of adverse reactions in 104 intravenous immunoglobulin infusions (IntraglobinTMF) given to 13 patients suffering from several immunodeficiency and autoimmune diseases over 15 months in King Hussein Medical Center. The severity of the reaction was classified as mild, moderate and severe. Transmission of infections such as human immunodeficiency virus, hepatitis B, and C was monitored. RESULTS: The total number of reactions documented was 16 out of 104 (14.5%) infusions and all resolved without medical aid. Excluding those reactions in which a predisposing factor was identified, the overall rate was 6%. CONCLUSION: This study has shown that the overall reaction rate in patients infused with intravenous immunoglobulin at hospital is low. However, establishment of specialized staff will decrease the adverse reactions considerably. We advise the care-taking staff to elicit the predisposing factors like infection and avoid infusion until antibiotics have been started for 24-48 hours. We found that strict application of manufacturer infusion protocols (IntraglobinTMF) will avoid reactions due to accelerating rate of infusion. Intravenous immunoglobulin is a useful life saving drug. It is safe if all precautions are taken into consideration.

Sertoli Cell Tumor of Testes in a Child with Peutz-Jeghers syndrome.

The Peutz-Jeghers syndrome (PJS) is known to be commonly associated with ovarian tumors. However, its association with testicular tumors is uncommon. We report an eight-year old boy who presented with gynecomastia and had Sertoli cell tumor of both testes with high aromatase activity. To the best of our knowledge, this is the seventh such case being reported in the literature and the presenting manifestation in all seven was gynecomastia.