Prevalence of post-thrombotic syndrome following asymptomatic thrombosis in survivors of acute lymphoblastic leukemia.

BACKGROUND: Deep vein thrombosis (DVT) is a complication of treatment of acute lymphoblastic leukemia (ALL) in children but little is known about the long-term outcomes of these DVT. OBJECTIVE: To determine the incidence of post-thrombotic syndrome (PTS) in (i) children with ALL diagnosed with asymptomatic DVT using radiographic testing and (ii) an unselected group of ALL survivors. METHODS: Cross-sectional study in two populations. Group I comprised children in the Prophylactic Antithrombin Replacement in Kids with ALL treated with L-Asparaginase (PARKAA) study diagnosed with DVT by radiographic tests. Group II consisted of non-selected childhood ALL survivors <21 years. PTS was assessed using a standardized scoring sheet. RESULTS: Group I: 13 PARKAA patients (median age 12 years) were assessed, and 7 had PTS (54%; 95% CI, 25-81). All patients had collaterals, three also had increased arm circumference. Group II: 41 patients (median age 13 years) with a history of ALL were enrolled, and 10 had PTS (24%; 95% CI, 11-38). All patients had collaterals; five also had increased arm circumference. CONCLUSION: There is a high incidence of PTS in survivors of childhood ALL with radiographically diagnosed asymptomatic DVT. A significant proportion of ALL survivors develop PTS, indicating previously undiagnosed DVT.

Cognitive and academic late effects among children previously treated for acute lymphocytic leukemia receiving chemotherapy as CNS prophylaxis.

OBJECTIVE: Examine cognitive and academic late effects among children and adolescents who had received central nervous system (CNS) prophylactic chemotherapy alone for acute lymphocytic leukemia (ALL); none had received whole brain radiation therapy (RT). METHOD: Subjects included 47 children and adolescents from 5 to 22 years of age who were treated on the same protocol and had been off treatment from 2 to 7 years at the time of assessment. RESULTS: As a group the survivors displayed generally average performance on measures of cognitive and academic abilities, although they differed from normative means on tests of nonverbal skills. Girls performed more poorly than the normative sample on nonverbal tasks, while no differences were found for boys. Age at diagnosis and time off treatment were not significantly associated with cognitive and academic functioning for survivors of this particular chemotherapy-only protocol. CONCLUSIONS: Data were interpreted to support generally modest potential late effects in specific areas for children and adolescents surviving ALL. These findings suggest a need for monitoring nonverbal cognitive skills for childhood survivors of ALL, particularly for girls.

Transient erythroblastopenia of childhood. Prospective study of fifty patients.

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.

Transient erythroblastopenia in a child with Kawasaki syndrome: a case report.

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.

Acute myocardial infarction complicating prothrombin complex concentrate therapy in an 8-year-old boy with hemophilia A and factor VIII inhibitor.

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.

Sonographic abnormalities of the thyroid gland in longterm survivors of Hodgkin disease.

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.

Magnetic resonance imaging in the diagnosis of adolescent colorectal carcinoma.

A 14-year-old girl presented with a large abdominal mass, thought to be an ovarian tumor on the basis of clinical and sonographic findings. MRI correctly suggested a primary colonic neoplasm. At operation, ovarian involvement was found to be secondary to metastatic signet ring cell adenocarcinoma of the colon, an extremely rare malignancy in this age group. Low index of suspicion for this tumor in children and adolescents results in advanced disease at diagnosis and poor prognosis. Early imaging with MRI is recommended when the nature of pelvic disease in children remains obscure.