Failure to attend appointments and loss to follow-up: a prospective study of patients with malignant lymphoma in Riyadh, Saudi Arabia.

Failure to attend medical appointments (No Show) and loss to follow-up (LTFU) among patients with cancer can adversely affect their treatment and eventual outcome. In a 3-year prospective study of 199 patients with malignant lymphoma, all of those with No Shows were contacted, and reasons given for No Shows were categorized. Of the 340 No Shows, 34.1% were due to hospital-based communication problems, 17.6% to errors in patient communication with the hospital, 7.4% to transportation problems and 16.5% to other personal reasons. Almost one quarter (24.4%) of the patients were not contactable. Reasons for No Show in all categories were instructive as to patients' attitudes to treatment. Nineteen (12.2%) of the 156 patients who had not died in the 3-year follow-up period were identified as LTFU. These 19 LTFU patients accounted for 77 (22.6%) of all No Shows. The data indicate that LTFU in this cohort is significantly less frequent than in a prior cohort followed up for 3 years from 1997 to 1998. These findings suggest that some causes of No Show can be addressed, and individuals are identified as at particular risk for No Show and ultimately LTFU. This study points out that pre-emptive strategies to reduce No Shows may be feasible and efficacious.

Allogeneic stem cell transplantation in patients with ß-thalassemia: King Faisal specialist hospital and research centre experience.

From January 1998-July 2006, 62 stem cell transplantation (SCT) were performed on 60 patients with beta-thalassemia from HLA-related match donors. The overall survival (OS) and event free survival (EFS) for all patients were 94 and 77%. The outcome of allogeneic SCT in our experience is satisfactory with OS 92% and EFS 77%. Transplantation at a young age and when the disease is mild offers the best outcome. More advanced disease is associated with higher rate of rejection and severe graft versus host disease.

A human T cell lymphoma secreting an immunoglobulin E specific helper factor.

An 8-yr-old nonallergic girl with non-Hodgkin's lymphoma had markedly elevated serum IgE at presentation (greater than 10,000 IU/ml), negative skin tests to a battery of 24 common allergens, and no evidence of parasitic infestation. Serum levels of IgG, IgA, and IgM were normal. Remission after cytotoxic chemotherapy was accompanied by a marked reduction in serum IgE levels (to less than 200 IU/ml) with no change in the level of serum IgG, IgM, or IgA. Recurrence of the lymphoma 7 mo after remission was accompanied by an isotype specific rise in serum IgE (to 3,850 IU/ml). Isoelectric focusing revealed that the IgE was polyclonal. Phenotypic analysis of the lymphoma obtained during relapse revealed all (greater than 98%) cells to be T3+, T4+, and T8+. Incubation of lymphoma cells with human myeloma IgE followed by immunosorbent purified fluorescein tagged goat anti-human IgE (anti-IgE PS-adsorbed over IgE ADZ) stained 25% of the cells. In contrast, less than 1% of the cells were stained after incubation with human IgG followed by fluorescein conjugated goat anti-human IgE. Supernatants from lymphoma cells (5 X 10(6)/ml, 48 h) enhanced IgE production in B cells derived from four patients with allergic rhinitis (mean +/- SD picograms per milliliter of net IgE 930 +/- 320 in unstimulated cultures versus 2,450 +/- 650 in cultures stimulated with lymphoma supernatants; P less than 0.01) but did not induce IgE synthesis in B cells from two normal subjects that synthesized no IgE spontaneously. Lymphoma supernatants failed to enhance IgG synthesis by B cells of both allergic and nonallergic subjects. These results indicate that a T cell lymphoma comprised of cells bearing Fc receptors for IgE with a phenotype characteristic of immature T cells (i.e., T3+, T4+, T8+) exhibited IgE specific helper function. This lymphoma may represent the monoclonal expansion of a subpopulation of IgE specific helper T cells.

Aspiration cytology of retinoblastoma: light and electron microscopic correlations.

A series of 16 cases of retinoblastoma diagnosed by fine-needle aspiration biopsy (FNAB) and confirmed by histologic examination is reviewed, and the salient cytomorphologic features are described. Two types of cells were encountered in the aspiration smears; type I cells were undifferentiated while type II cells showed more differentiation and frequently revealed cytoplasmic processes that are probably indicative of early photoreceptor differentiation. Flexner-Wintersteiner rosettes characteristic of retinoblastoma were found in 10 of 16 cases. These findings were further correlated with ultrastructural examination of the tumors in nine cases. It is concluded that the presence of rosettes and type II cells with cytoplasmic processes are the two features that are most helpful in the FNAB diagnosis of retinoblastoma.

Aspiration cytology of neuroblastoma: light microscopy with transmission and scanning electron microscopic correlations.

Fine-needle aspiration biopsies from three patients with neuroblastoma were studied by light microscopy, and the morphologic findings were correlated with those from transmission and scanning electron microscopy. Light microscopic examination of the aspiration smears from all three cases revealed small and large round cells with variable numbers of intertwining cytoplasmic processes. Transmission electron microscopy confirmed the light microscopic finding of cytoplasmic processes; in addition, it revealed the presence of other diagnostic morphologic features, including neurosecretory granules, microtubules, and synaptic cell junctions. Scanning electron microscopy demonstrated that the tumors were composed of a mixture of undifferentiated round cells and more differentiated cells with long cytoplasmic processes. The morphologic spectrum of these processes and their interrelationships with one another and with other cells could be studied in detail. These findings indicate that scanning electron microscopy may be used effectively in the morphologic evaluation and pathologic diagnosis of neuroblastoma.

Fine-needle aspiration biopsy of Langerhans histiocytosis (histiocytosis-X).

A series of 14 fine-needle aspiration biopsies (FNAB) from histologically proven cases of histiocytosis-x (Hx) were reviewed. The smears revealed a variable mixture of Langerhans cells, eosinophils, macrophages, polymorphonuclear cells, and giant cells. Based on the predominant cells present, the cases were further categorized as Langerhans cell predominant (nine cases), eosinophil predominant (two cases), and macrophage predominant (three cases). Langerhans cells were usually polygonal without significant evidence of phagocytosis and frequently contained indented nuclei. In three cases, several Langerhans cells showed prominent dendritic processes. Electron microscopy in 10 cases revealed Langerhans cells with varying degrees of morphologic differentiation. All cases studied revealed Birbeck granules. These findings indicate that FNAB may be an effective technique for diagnosing Hx.

Aspiration cytology of Wilms' tumor: correlation of cytologic and histologic features.

We examined the cytomorphologic features of fine-needle aspiration biopsy (FNAB) specimens from 23 Wilms' tumor patients. The findings were correlated with histopathologic patterns from these tumors. The study revealed a close resemblance between the cytologic and histopathologic appearance of various cellular elements in Wilms' tumors. The major cellular patterns seen in Wilms' tumor include blastemal cells, blastemal cells with epithelial differentiation, blastemal cells with tubular differentiation, and stromal elements. It is hoped that recognition of these cellular components in aspiration smears will be helpful in establishing an FNAB diagnosis of Wilms' tumor.

Small round cell tumor with divergent differentiation: cytologic, histologic, and ultrastructural findings.

A case study of desmoplastic small round cell tumor with divergent differentiation in a 13-yr-old boy is presented. Clinical, cytologic, histologic, immunohistochemical, and ultrastructural findings are reported. There was a close correlation between the various histologic and cytologic patterns. Immunohistochemical studies revealed strong positivity of many of the tumor cells for cytokeratin, vimentin, and desmin. Ultrastructurally, some of the tumor cells contained aggregates of cytoplasmic intermediate filaments. Fine-needle aspiration biopsy may suggest the diagnosis of desmoplastic small round cell tumor. Confirmation of the diagnosis may be provided by immunohistochemistry and electromicroscopy.

Acute thrombocytopenic purpura and poststreptococcal acute glomerulonephritis in a child.

The occurrence of acute idiopathic thrombocytopenia purpura in association with poststreptococcal glomerulonephritis in a 5 1/2-year-old boy is described. This association has been reported only in two cases in the literature. We are reporting the first biopsy-proven confirmation of this association.

Histiocytosis-X in Saudi children.

A retrospective analysis of 21 Saudi children with Histiocytosis-X were reviewed. 13 were males, 8 were females, with a ratio of 1.62:1. Five had unifocal disease while 16 were with multifocal disease. Bone involvement was seen in 19 patients (90.4%), and three patients had organ dysfunction. One patient had family history of Histiocytosis-X. Follow-up was from 1-10 years (Median 3 years). Three patients died, three had recurrences and eight patients had various disabilities (38%). Overall disease free survival was 84.2 percent.

Chediak-Higashi syndrome: Report on five Saudi Arab children and review of the literature.

Five cases of the Chediak-Higashi Syndrome (CHS) among Saudi Arab children were diagnosed between June 1978 and December 1990. All patients were males, ages 18 months to ten years, born to first degree consanguineous parents. All showed the typical somatic and laboratory characteristics of this syndrome with characteristic hyperpigmented irides in four patients. Three patients were in the accelerated phase, two of whom responded to a combination chemotherapy (vincristine and prednisone) and one improved after splenectomy. All patients died before they passed the first decade of their lives. This report is the first detailed description of CHS among Arab children in the Arabian peninsula.

Pediatric cancer: The King Faisal Specialist Hospital and Research Centre experience.

Between January 1976 and December 1993, a total of 3291 children with cancer were treated at King Faisal Specialist Hospital and Research Centre (KFSH&RC). Males accounted for 60.7% and females 39.3%, with a ratio of 1.5:1. The peak age was two to five. The three most common malignancies were leukemias (26.2%), lymphomas (21.3%), and central nervous system (CNS) tumors (15.3%). This report presents some of the epidemiologic data related to the largest number of children with malignancy treated in a single institution in Saudi Arabia.

Large unilateral chest tumors in children.

When large unilateral chest tumors are hidden by pleural fluid collections, or when there is a completely radiopaque hemithorax, ultrasonography is an efficient means of identifying and characterizing a mass. Narrowing of the carinal angle in patients with mediastinal shift is helpful in identifying large intrathoracic tumors that could be mistaken for, or hidden by, large pleural effusions. Of 11 large tumors in children, seven were Ewing's sarcomas, two were metastatic sarcomas, one was a lymphoma, and one was an undifferentiated small cell tumor of unknown origin. The rib lesions of Ewing's sarcoma were seen clearly on chest radiographs in all patients with that disease.

Partial albinism, immunodeficiency, and progressive white matter disease: a new primary immunodeficiency.

We studied 12 children who presented with a recently recognized syndrome. The salient features of this new syndrome were recurrent fever; hepatosplenomegaly; pancytopenia; blond, golden to silvery gray hair; hypopigmented skin, progressive white matter demyelination; and early death. Seven patients died, four with severe central nervous system (CNS) involvement, and three with bone marrow failure and sepsis. Cutaneous anergy to recall antigens was present in all patients. Other immunological abnormalities were poor antibody responses; deficient T-cell responses to phytohemagglutinin (PHA), concanavalin A (Con-A), and allogeneic lymphocytes; hyperresponsiveness of B lymphocytes to pokeweed mitogen; and variable phagocytic defects. Histopathologic examination of the hair and skin biopsies showed characteristic distribution of melanin with melanocytes present in normal numbers but with fewer short dendritic processes. Langerhans' cells were present in normal numbers in some patients and sparse in others. This syndrome seems to cluster into two tribes from two different geographical areas in the Arabian Peninsula. In the eight families studied, 12 other siblings and close relatives were found to be affected. The mode of inheritance in this syndrome is that of an autosomal recessive pattern. We propose the term "PAID syndrome" to identify patients with the above features.

Primary unilateral renal lymphoblastic lymphoma.

A 4-year-old boy presented with a right-sided mass. A lobulated tumour in the right flank was felt on palpation, and radiological investigation revealed a solid, multinodular intrarenal tumour. On biopsy the tumour was found to be a lymphoblastic lymphoma.

Assessment and comparison of intraoperative and postoperative pain associated with NovaSure and ThermaChoice endometrial ablation systems.

STUDY OBJECTIVE: To assess and compare intraoperative and postoperative pain associated with NovaSure impedance-controlled endometrial ablation system and ThermaChoice system. DESIGN: Prospective, international, multicenter, double-arm study (Canadian Task Force classification II-1). Setting. Academic medical centers and private offices. PATIENTS: Sixty-seven premenopausal women with menorrhagia. INTERVENTION: Endometrial ablation with either the NovaSure (37 women) or ThermaChoice (30) system. NovaSure-treated patients received no endometrial pretreatment; those treated with ThermaChoice received the recommended 3-minute suction dilatation and curettage. MEASUREMENTS AND MAIN RESULTS: Standard pain measurement instruments (visual analog scale, numeric rating scale) were used to assess intraoperative and postoperative pain. Serum levels of prostaglandin-F(2alpha) were measured before and 5, 30, and 60 minutes after the procedure. Patients treated with the NovaSure system reported statistically significantly lower intraoperative and postoperative pain than those treated with the ThermaChoice system (p <0.0001). Procedure time was statistically significantly shorter with the NovaSure system (p <0.0001). Prostaglandin-F(2alpha) values did not differ statistically between groups. CONCLUSION: The NovaSure system is associated with statistically significantly lower intraoperative and postoperative pain than ThermaChoice system, and endometrial ablation with NovaSure could become an office-based procedure.

Primary T-cell lymphoma of the brain in children: a case report and literature review.

Described here are the clinical features and results of treatment in a 10-year-old Saudi Arabian girl with primary T-cell lymphoma of the central nervous system. At presentation the patient had nystagmus and ataxia. The diagnosis was established by tissue biopsy obtained from the cerebellum. Therapy included cranio-spinal irradiation and combination chemotherapy of a systemic high dose of methotrexate, cytosine, arabinoside, and L-asparaginase. Remission was obtained easily but was interrupted by a local intracranial relapse 57 months after diagnosis (37 months after cessation of therapy; at present the patient is still alive and receiving palliative treatment). This report is warranted because of the rarity of this condition in children.

Aspiration cytology of Ewing's sarcoma. Light and electron microscopic correlations.

Fine-needle aspiration biopsy specimens from 15 histologically proven cases of Ewing's sarcoma of the bone were studied by both light and electron microscopy. Large glycogen deposits in the Ewing's sarcoma cells could be easily recognized in smears stained routinely with Diff-Quik (American Scientific Product, McGraw Park, IL). These deposits appeared as punched-out clear spaces in the cytoplasm. Ultrastructurally the tumor cells could be divided into chief cells and dark cells. The chief cells were further categorized based on their nuclear morphologic features into cleaved, noncleaved, and intermediate cells. The dominant cell was noncleaved in eight, cleaved in three, and intermediate in four cases. All five tumors with abundant glycogen were composed predominantly of noncleaved cells. The tumor cells in aspiration material appeared to be irregular in shape, and some had cytoplasmic processes. In one tumor these processes were quite prominent; this caused some difficulty in differentiation from neuroblastoma.

Tru-cut needle biopsy of abdominal tumors in children: a safe and diagnostic procedure.

Fifteen children with inoperable abdominal malignancies underwent percutaneous biopsy. Both fine needle aspiration (FNA) and core biopsies using a cutting needle were obtained. In 5 of the 15 specimens obtained by FNA, the material obtained showed malignant cells but did not allow a precise diagnosis to be made. In a sixth case, the diagnosis made on a FNA specimen proved to be incorrect. In contrast, all 15 specimens obtained with a cutting needle were diagnostic. No complications were observed following these procedures. Core biopsies appear to be a safe and reliable means of obtaining tissue for diagnosis in children with inoperable solid abdominal masses.