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Lymphomatoid papulosis (LyP) is a benign, self-healing, papular eruption that can wax and wane over the course of time. Transformation to T-cell lymphoma has been well documented in 10% to 20% of adults with LyP, but there are have been no cases reported in patients younger than age 26 years. We describe the first pediatric patient, a 16-year-old girl, who had clinical features of LyP and concurrently was found to have a lesion diagnosed as Ki-1+ anaplastic large cell lymphoma. After treatment with chemotherapy, she has been in continuous remission for 16 months.

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The residual antibiotic concentration of vancomycin (2 mg/ml)- or ceftazidime (2 mg/ml)-heparin solutions instilled in ports in pediatric hematology-oncology patients 1 to 34 days earlier was measured. Antibiotic concentrations of > or = 100 microg of either antibiotic per ml persisted for at least 21 days. For treatment of lumenal port infections, antibiotic-heparin dwell times of > or = 2 weeks may be appropriate.

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Thrombocytopenia absent corpus callosum, characterized by refractory thrombocytopenia, agenesis of the corpus callosum, hypoplastic cerebellum, abnormal facies, and developmental delay, represents a relatively newly described clinical entity. An 18-month-old girl with agenesis of the corpus callosum, hypoplasia of the cerebellar vermis, hypotonia, and severe developmental delay presented with thrombocytopenia. She had a distinctive facies with microcephaly, broad nasal root with upturned nose, small upper lip, and micrognathia. A bone marrow aspirate and biopsy showed normal cellularity with dysplastic megakaryocytes. Thrombocytopenia absent corpus callosum is compared with other conditions characterized by congenital non-immune thrombocytopenia.

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Background: As the role of PET-FDG imaging is being established in the staging and monitoring of response to therapy in children with lymphoma, we encountered a case of an infection common in adolescence that may present with lymphoma-like signs and symptoms.Methods: A 13-year-old previously healthy male presented with a left neck mass associated with weakness, fatigue, intermittent fevers and weight loss. He was then referred to the hematology/oncology department with a working diagnosis of lymphoma. The total wbc count was 5920/cu mm with 75% lymphocytosis without atypical lymphocytes. ESR was 20 mm. Serologic analysis for EBV, CMV, toxoplasmosis and hepatitis was also performed. The chest x-ray was normal. CT scan demonstrated multiple enlarged lymph nodes in both right and left jugulodigastric and spinal accessory chains; the largest mass within the left spinal accessory chain had focal necrosis within it. There were no enlarged mediastinal or axillary nodes. The spleen was massively enlarged and the splenic index was 924 (normal for age = 744).Results: FDG imaging showed intense uptake in both cervical regions, the mediastinum and in the enlarged spleen. The results of the Monospot test and the EBV panel which were both positive, were available 3 & 5 days later. Based on these serologic results, the history, physical findings and the negative chest x-ray, the final diagnosis was infectious mononucleosis.Conclusion: Despite availability, ease of performance and sensitivity of FDG imaging, this case illustrates the importance of clinical, hematologic and serologic assessment of disease prior to FDG imaging.

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The efficacy of antibiotic treatment of port-associated bloodstream infection without device removal has not been systematically studied. We analyzed the outcome of 43 consecutive port-associated bloodstream infections in pediatric hematology-oncology patients. Etiologies included Staphylococcus epidermidis (30) and Staphylococcus aureus (6). Antibiotics were given through the port for a median of 11 days. Four ports were removed within 72 hours. In 36 (92%) of the remaining 39 episodes, there was a response to antibiotic therapy (defervescence and negative blood culture). In 78% of episodes in which there was a response (excluding two in which the catheters were removed because of mechanical problems), the infections were cured without port removal. Two of the four relapses were cured with a second course of antibiotics. The cure rate was 92% for S. epidermidis infections and 67% for S. aureus infections. Thus, the majority of port-associated bloodstream infections in pediatric hematology-oncology patients can be cured without device removal.

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PURPOSE: In this report, the Pediatric Oncology Group (POG) experience with lymphocyte predominant Hodgkin Disease (LPHD) in children is reviewed. MATERIALS AND METHODS: From 1984-1993, the POG conducted 3 clinical trials for advanced stage HD and 2 for early stage HD. There were 26 cases of LPHD in 613 patients in these trials. Patients' ages ranged from 3.1-17.8 years (mean of 12.9 years). There was a marked male predominance. RESULTS: Histologic subtypes were 17 nodular, 8 diffuse pattern; 1 was indeterminant. The sites involved at diagnosis were primarily the peripheral lymph nodes. Fourteen patients had stage (S) I disease; 9 had SII; 3 had SIII; there was no SIV disease. Only 4 of 26 patients had B symptoms. All 26 patients achieved complete remission, 10 with radiotherapy, 6 with chemotherapy and 10 with combined modality therapy. Treatment was not uniform since patients were registered on different protocols. Event-free survival after 5 years was 86.5 percent. Two patients developed and succumbed to large cell, T-cell type, non-Hodgkin lymphoma (NHL). CONCLUSIONS: Optimal treatment for LPHD should focus on efforts to limit the risk of second malignancy.

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OBJECTIVE: To determine whether the immune systems of long-term survivors of acute lymphoblastic leukemia (ALL) have persistent immune defects after Berlin-Frankfurt-Münster (BFM) treatment. STUDY DESIGN: We evaluated the cellular and humoral immune responses of 13 children with ALL in complete remission and off modified protocol treatment for 2 or more years. All patients had received complete immunizations for measles, mumps, rubella, and poliovirus before ALL developed. They were challenged with Haemophilus influenzae type B (Hib) and Pneumococcus vaccines after baseline serum samples were obtained. We also determined in vivo humoral immune responses to bacteria and viruses that cause common pediatric diseases. RESULTS: Compared with age-matched control subjects, the long-term survivors of ALL had a significant difference in the presence of protective antibodies to measles (p < 0.0001) and polioviruses (p < 0.0001) in their baseline sera; more than half had no protective antibodies to one or more previously administered vaccines or related infections. Most produced protective concentrations of specific antibody after reimmunization, but some were repeatedly unable to make protective antibodies, or mount a normal antibody response, despite natural disease and/or revaccination. Four children had significant infections. CONCLUSIONS: Long-term survivors of ALL who had BFM treatment may have persistent immune defects with respect to common childhood bacterial and viral diseases they previously had, or vaccines they received.

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PURPOSE: To prospectively evaluate 50 patients with transient erythroblastopenia of childhood (TEC) at a single institution in order to compare those patients presenting with reticulocytopenia (group I) with those presenting in the recovery phase with reticulocytosis (group II); to further describe the clinical course of this common pediatric hematological disorder in a large number of patients, particularly the effect on the neutrophils; and to review the available literature regarding this disorder. PATIENTS AND METHODS: Fifty patients presenting to the Children's Hospital from September 1983 to September 1991 were prospectively evaluated. Those patients with a reticulocytosis and in recovery at the time of diagnosis were included and compared with those with reticulocytopenia. All patients were followed through complete recovery. RESULTS: Thirty-six patients were reticulocytopenic (group I) and 14 had a reticulocytosis (group II). There was a high incidence of neutropenia (64%) in both groups and the resolution of this neutropenia was variable in relation to the resolution of the anemia, with 44% having the lowest ANC before, 9% simultaneous with, and 47% after the peak reticulocyte count. CONCLUSIONS: Our experience with a large group of patients with TEC suggests that neutropenia is an integral part of this disorder, and its recovery has no relation to the recovery of the anemia. A significant number of patients are described in the recovery phase for the first time, and this clarifies this group of patients in order to aid in their diagnosis, particularly in the differentiation from a hemolytic process. Some previously described associations of TEC are not supported in this study of a large number of patients.

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PURPOSE: We describe for the first time the case of a child with Kawasaki syndrome and associated transient erythroblastopenia. PATIENTS AND METHODS: A 5 1/2-month-old infant with Kawasaki syndrome as evidenced by lymphadenopathy, fever, rash conjunctival injection, and extremity changes had associated anemia and reticulocytopenia requiring transfusion and thrombocytopenia. Bone marrow aspiration was consistent with a transient erythroblastopenia. She developed cardiac aneurysms despite therapy with i.v. immunoglobulin and aspirin. RESULTS: The anemia and reticulocytopenia resolved with normalization of her hemoglobin. The platelet count increased and she developed a thrombocytosis characteristic of this clinical entity. She completely recovered without recurrence of either the anemia or reticulocytopenia. CONCLUSIONS: We speculate that the cause of the erythroblastopenia and thrombocytopenia is an inflammatory insult of Kawasaki syndrome on the bone marrow and its various lineages causing the myriad hematological abnormalities now associated with Kawasaki syndrome. It is possible that the i.v. immunoglobulin aids in neutralizing the triggering agent and therefore shortening the duration of the marrow insult.

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Cord blood is a recently recognized source of hematopoietic stem cells. It can be employed successfully to reconstitute hematopoiesis following allogeneic transplantation. One current drawback of cord blood as a treatment has been a risk of transfusion reactions attributable to ABO blood group mismatch. Removal of red cells from the cord blood has led to reduction of the stem cells by 30-50%. In this paper we report red cell depletion by a method that employs 3% gelatin to effectively sediment the erythrocytes and selectively deplete red cells but permits 94% recovery of nucleated cells and enrichment of colony-forming cells by granulocyte-macrophage colony-forming units, erythrocyte burst-forming units, and granulocyte-macrophage-megakaryocyte colony-forming units in the cord blood preparation. This technique has been employed in our study to remove red cells from the cord blood of a male infant delivered by cesarean section, which has permitted treatment of a female sibling suffering from leukemia. The recipient was 8 years old and weighted 36.7/kg. Complete HLA identity between the two siblings was established. A cord blood cell transplant of cryopreserved and later thawed cells (4 x 10(7) nucleated cells per kilogram) was administered to the patient after intensive myeloablative chemotherapy. The patient exhibited a prompt hematologic recovery (absolute neutrophil count > 500 by day 31, 100% male cells in bone marrow and peripheral blood by day 25) and has experienced a 13-month disease-free survival to date.(ABSTRACT TRUNCATED AT 250 WORDS)

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PURPOSE: An 8-year-old boy with mild hemophilia A and inhibitors developed an acute myocardial infarction during treatment with prothrombin complex concentrates. CONCLUSIONS: This rare complication warrants restriction of dosage and length of treatment with these products to the recommended guidelines. It also appears that noninvasive cardiac monitoring of these patients is beneficial.

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Current imaging modalities are accurate in establishing the diagnosis and extent of thoracic Hodgkin disease. After treatment, however, it is extremely difficult to differentiate potential residual active neoplastic disease from scar tissue, or identify early recurrence. We evaluated the contribution of MRI in the assessment of the response to treatment of thoracic Hodgkin disease in the assumption that scar formation would be characterized by low signal intensity in all pulse sequences, whereas active tumor should maintain a degree of high signal intensity on T2-weighted images. In 47 occasions (23 patients) both CT and MRI were able to identify correctly active disease, but had low specificity in confirming remission because of residual tissues masses. High signal intensity on T2-weighted MR images often persisted despite remission, probably because of edema, necrosis, granulation or other factors. MRI was somewhat more specific than CT and may be quite valuable to confirm remission in patients with residual masses that no longer appear hyperintense on T2 after treatment.

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We studied the thyroid gland in 18 long term survivors of Hodgkin Disease, all of whom received radiation therapy (2400-4000 cGy, mean 3434). Evaluation included clinical examination, thyroid function tests, ultrasound, as well as selective use of nuclear scintigraphy. The time interval post radiation was 1-16 years (mean 6.4 years). Clinical evaluation and thyroid function tests were insensitive in detecting abnormalities and most nodules were non palpable. Ultrasound detected abnormalities in 16 patients (89%) including diffuse atrophy (n = 6), solitary nodules (n = 4), multiple nodules (n = 5) and gland heterogeneity with calcification in one patient. Cold nodules on nuclear scintigraphy (n = 4) were subjected to biopsy. Multiple foci of papillary carcinoma were found in one patient. There was a tendency for nodules to increase in number as the post radiation interval also increased. We recommend frequent sonographic evaluation and early suppression of thyroid stimulation in an attempt to arrest the development of neoplastic changes.

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Since thrombotic phenomena have important roles in the vasoocclusive manifestations in sickle cell disease (SCD), protein C (PC) was determined in 32 children with SCD during steady state and vasoocclusive crisis (VOC) and in controls. Children with SCD during steady state had significantly lower PC levels as compared to controls. During VOC there was marked decrease in PC as compared to levels during steady state, and these levels increased to initial levels or higher with clinical improvement. It is postulated that the decreased levels of PC in SCD are probably secondary to increased consumption as well as decreased production because of altered liver function. This data suggests that decreased levels of PC may increase the risk of thrombosis in these patients.

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The most significant clinical manifestations of sickle cell disease (SCD) are those associated with CNS involvement. This disease is systemic in character, with the tissues of the CNS being the most sensitive to even temporary anoxia. For this reason, the incidence of peripheral and central auditory dysfunction in patients with SCD may be greater than in the "normal" population. We examined the peripheral auditory function of 54 patients with homozygous sickle cell disease and the central auditory function of 28 of these patients. Results were compared with a control population. Peripheral hearing loss was found in 11% (6) of the SCD population. Central auditory testing indicated that 46% (13) of the sickle cell population had mild central auditory dysfunction not involving Heschl's gyrus. This study suggests a higher than usual incidence of peripheral and central auditory impairment in sickle cell disease.

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Newborn rats born to iron deficient mothers (IDM) were found to have significantly lower hemoglobin, sucrase, lactase and maltase levels compared to control newborn rats. Rats born to IDM and nursed by IDM, when sacrificed at 21 days of age, had statistically significantly lower hemoglobin, serum iron, sucrase, lactase and maltase levels compared to control rats. Rats born to IDM, but nursed by iron sufficient mothers (ISM) and sacrificed at 21 days of age, had hemoglobin, serum iron and sucrase levels compared to control rats whereas lactase and maltase were not corrected by 21 days of nursing by ISM. Rats burn to IDM and nursed by either IDM or ISM for 21 days were given intramuscular iron dextran and placed on iron sufficient diet (ISD) for 7 days. These animals experienced correction of the hemoglobin, serum iron, sucrase and maltase levels compared to control rats, whereas intestinal lactase was not corrected by 7 days of ISD and intramuscular iron. Rats born to ISM, nursed by IDM and sacrificed on day 21 had significantly lower hemoglobin, serum iron and intestinal lactase levels compared to control rats. Rats both to ISM and nursed by IDM were given intramuscular iron dextran on day 21 and placed on an ISD from day 21-28. These animals had a return in hemoglobin, serum iron, sucrase and maltase levels comparable to control rats. Rats born to and nursed by ISM and maintained on an iron deficient diet from day 21-84 had significantly lower hemoglobin, serum iron, sucrase, lactase and maltase levels compared to control rats. Rats born to and nursed by ISM, maintained on iron deficient diet from day 21-84, and then given intramuscular iron dextran on day 84 and maintained on an ISD until day 92, experienced correction of the hemoglobin, serum iron and lactase levels compared to control rats. Intramuscular iron and 7 days of ISD did not correct the sucrase and maltase levels in these rats. Lactose tolerance tests in iron deficient rats showed flat curves compared to controls. After iron treatment, lactose tolerance curves returned to control values. Iron deficiency in rats in utero, during the nursing and postweaning period causes, in addition to anemia, a reduction in jejunal disaccharidase activity because of an alteration in the enzymes of the brush border membrane. Varying degrees of reduction and response of certain disaccharidases to iron treatment are dependent on the time of iron deprivation in relationship to the intra-uterine and postnatal development of the digestive and absorptive functions in the small intestine. Alterations in the levels of disaccharidases demonstrated in this paper represents another aspect of the spectrum of biochemical effects of iron deficiency.

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