Concurrent t(4;11) and t(1:19) in a Pediatric Patient with B-Lymphoblastic Leukemia/Lymphoma (B-ALL): A Case Report and Review of the Literature.

We herein present the case of a pediatric patient with B-lymphoblastic leukemia (B-ALL) with MLL gene rearrangement associated with the t(4;11)(q21;q23). Complete remission was achieved with standard B-ALL-directed chemotherapy and whole brain radiation. The patient subsequently relapsed and cytogenetic assessment revealed an additional acquired t(1;19)(q23;p13) associated with the TCF3-PBX1 fusion. After reinduction chemotherapy with a relapse B-ALL protocol the patient achieved disease remission; however, he developed respiratory complications and died. This represents a unique case as these two translocations have never been described concurrently in pediatric acute leukemia patients.

Current difficulties and recent advances in bypass therapy for the management of hemophilia with inhibitors: a new and practical formulation of recombinant factor VIIa.

Bypassing agents are the mainstay of treatment for patients with hemophilia with high-titer inhibitors. Whereas the availability of these agents has greatly advanced the management of bleeding episodes in this population, timely administration of bypassing agents continues to be hampered by a number of practical limitations, including the need for refrigerated storage of the agent and its reconstitution at room temperature prior to administration, among others. In this review, the importance of early treatment of bleeds and factors that influence this more timely therapeutic approach are highlighted, together with the advantages offered by the use of a new formulation of recombinant activated factor VII that permits improved storage and portability, potentially optimizing timely bypassing agent administration.

The meaning of surviving cancer for Latino adolescents and emerging young adults.

Adolescent and young adult (AYA) survivors of cancer are an understudied population with unique developmental and medical needs that extend well beyond their active treatment. Survivors diagnosed as AYAs may experience both physical and emotional late effects. In particular, the experiences of Latino cancer survivors have not been explored. The purpose of this study was to conduct interviews with AYA Latino cancer survivors to inform professionals working with these survivors. A hermeneutic phenomenological approach was selected based on the focus on experiences and meanings of Latino adolescents' cancer survivorship. Phenomenology allows for understanding the subjective meaning and lived experience of populations that are understudied or marginalized. In-depth interviews were conducted with participants. Enrolled in the study were Latino AYAs between the ages of 14 and 21 years, after treatment. Interviews revealed 7 themes regarding the experience and meaning of survivorship for this population: gratitude, humor/positive attitude, empathy for younger children with cancer, God and faith, cancer happens for a reason/cancer changed my life, familial support, and staff relationships. Latino AYA cancer survivors develop meaning out of unique cancer experiences. Programs need to be developed specifically to address Latino adolescents and young adult survivors of cancer.

Leydig cell tumor after treatment for Ewing's sarcoma.

Leydig cell tumors account for 3% of testicular tumors and have never been reported after treatment for Ewing's sarcoma. We report the unusual occurrence of a patient who developed a Leydig cell tumor of the testis 18 years after successful treatment for Ewing's sarcoma. Additional monitoring for second malignancies may become appropriate as long-term survival continues to improve for patients with Ewing's sarcoma.

Intracranial hemorrhage in immune thrombocytopenic purpura: a retrospective analysis.

PURPOSE: To ascertain characteristics of children with immune thrombocytopenic purpura (ITP) and intracranial hemorrhage (ICH). METHODS: The authors identified 75 published cases of ICH in children with ITP by review of the literature from 1954 to 1998. Data pertaining to the ICH was recorded for age, gender, time from diagnosis of ITP (to ICH), platelet count, head trauma or arteriovenous malformation, concomitant medications, associated infections, other bleeding manifestations, prior treatment, and outcome. RESULTS Sixty-two cases represented 6 months to 20 years of age; 65% of patients were female. The median time from the diagnosis of ITP to ICH was 32 days (range 0 days to 8 years). Fifty of 69 ICH cases (72%) occurred within 6 months of diagnosis, but only 7 (10%) occurred within 3 days of diagnosis. The platelet count was less than 10000/microL in 71.4% of the cases. Treatment prior to the ICH was primarily steroids but also included intravenous immune globulin (IVIG), splenectomy, and others (interferon, azathioprine, or vincristine). There was no difference in mortality of patients before (56%) or after (54%) 1980. CONCLUSIONS: A very low platelet count appears permissive but not sufficient for ICH to occur in children with ITP. ICH occurs more commonly in acute ITP but can occur years after diagnosis. A significant number of patients develop an ICH despite having already initiated steroid treatment of ITP.

Delayed diagnosis of retinoblastoma: analysis of degree, cause, and potential consequences.

OBJECTIVE: To assess the degree, cause, and consequence of delays from presenting signs to diagnosis of retinoblastoma. METHODS: A retrospective chart review was conducted of 64 consecutive patients who presented to the Memorial Sloan-Kettering Cancer Center with newly diagnosed retinoblastoma. Seven patients with a positive family history were excluded. RESULTS: The median times from presenting signs to diagnosis for patients with unilateral and bilateral disease were 1.5 and 2.25 months (range: 0--46), respectively; for those who presented with leukocoria and strabismus, median times were 1.5 (range: 0--46) and 2.5 months (range: 0--24). Parents noted the first signs in 75% of the cases. Seventy-seven percent delayed seeking treatment, and primary care physicians (PCPs) delayed referral in 30%. Only 3 patients were referred from PCPs solely for physical examination findings. No adverse consequence of delayed diagnosis could be established clearly, but a trend toward eye loss being associated with longer delays in patients with bilateral retinoblastoma was noted. CONCLUSION: Leukocoria and strabismus secondary to retinoblastoma are usually first recognized by relatives rather than PCPs. At routine visits, PCPs should inform parents about the importance of reporting eye abnormalities, and children whose parents complain of leukocoria (white, shiny, jello-like eye) should be referred promptly to an ophthalmologist regardless of whether an absent red reflex is appreciated.