Metachronous Bilateral Granulocytic Sarcoma of the Testis in a Young Adult: A Report of an Unusual Entity

Granulocytic sarcomas are rare tumors composed of neoplastic blood cells, typically occurring during the course of acute nonlymphoblastic leukemia or before its onset. We present a case of a 23-year-old young adult man with metachronous granulocytic sarcoma of the testis without hematologic manifestations who was diagnosed with granulocytic sarcoma (GS). The patient was treated with right orchiectomy but relapsed with a left testicular mass 16 months later when a left orchiectomy was performed. The patient has been free of disease for 13 months following the left orchiectomy. This case highlights a rare hematologic cancer that urologists and pathologists should be aware of since it can present as a testicular mass. Only 3 cases of testicular GS without an associated hematologic disorder have been described. To the best of our knowledge, our patient is the first reported case in the English literature of metachronous GS of the testis with no evidence of hematologic disorder.

Association of HLA -A, -B and -DRB1 alleles with hematological diseases in Vojvodina

Major histocompatibility complex (MHC) genes are involved in various mechanisms of pathogenesis and immunoediting of hematological diseases. This study aimed to investigate the association between HLA -A, -B and -DRB1 alleles with hematological diseases. In this study, we performed DNA-based HLA typing by polymerase chain reaction analysis with sequence-specific primers (PCR-SSP) to distinguish HLA-A, -B, and -DRB1 alleles. Eighty-two patients with hematological diseases (29 with acute lymphoblastic leukemia (ALL), 19 with acute nonlymphoblastic leukemia (ANLL), 5 with chronic myelogenous leukemia (CML), 2 with chronic lymphocytic leukemia (CLL), 9 with myelodysplastic syndrome (MDS), 9 with lymphomas (M.Hodgkin (HL) and non-Hodgkin (NHL)), 7 with aplastic anemia (AA) and 2 with multiple myeloma (MM)), were included in the study and compared with 111 healthy blood donors, residents from Vojvodina, evaluating the strength of the observed associations by measuring the aetiologic and preventive fractions. Among the alleles significantly associated with hematological diseases, HLA-A*24 showed an aetiologic fraction higher than those of HLA-A*26 and A*25 (RR=1.027, EF=1.233, RR=1.047, EF=1.141 and RR=1.213, EF=0.910).Negative association with significant preventive fraction was observed with HLA-B*18 and HLA-DRB1*11 alleles, with RR=0.400, PF=0.179 and RR=0.587, PF=0.176. Our results suggest that HLA-A*24, A*26 and A*25 as associated more frequently than other specificities with a hypothetical disease predisposing genes, may play a role in the pathogenesis of hematological diseases.

Protracted Intermittent Schedule of Topotecan in Children With Refractory Acute Leukemia: A Pediatric Oncology Group Study

PURPOSE: To determine dose-limiting toxicity (DLT) and maximum-tolerated dose (MTD) of a protracted, intermittent schedule of daily 30-minute infusions of topotecan (TPT) for up to 12 consecutive days, every 3 weeks, in children with refractory leukemia. PATIENTS AND METHODS: Forty-nine children were enrolled onto this phase I trial (24 with acute nonlymphoblastic leukemia [ANLL] and 25 with acute lymphoblastic leukemia [ALL]). TPT dosage was escalated from 2.0 to 5.2 mg/m2/d for 5 days and 2.4 mg/m2/d from 7 days to the same dose for 9 and 12 days in cohorts of three to six patients when no DLT was identified. TPT pharmacokinetics were studied in 33 children once or twice (first and last doses in patients who received TPT for > 7 days). RESULTS: Seventy assessable courses of TPT were administered to 49 children who had refractory leukemia. DLTs were typhlitis, diarrhea, and mucositis, and the MTD was 2.4 mg/m2/d for 9 days in this group of heavily pretreated children. In 33 patients, the median TPT lactone clearance after the first dose was 19.2 L/h/m2 (range, 9.4 to 45.9 L/h/m2) and did not change during the course. There were significant responses (one complete response [CR] and four partial responses [PR] in patients with ANLL and one CR and two PRs in patients with ALL), and all but one were at dosages of TPT given for at least 9 days. CONCLUSION: The MTD was 2.4 mg/m2/d for 9 days. Further testing is warranted of TPT’s schedule dependence in children with leukemia.