Myeloid Sarcoma after Allogenic Stem Cell Transplantation for Acute Myeloid Leukemia: Successful Consolidation Treatment Approaches in Two Patients

Myeloid sarcoma is an extramedullary (EM) manifestation (i.e., manifestation outside the bone marrow) of acute myeloid leukemia (AML); it is assumed to be relatively uncommon and can be the only manifestation of leukemia relapse after allogenic stem cell transplantation (allo-SCT). An EM sarcoma can manifest in any part of the body, although preferentially manifesting in immunological sanctuary sites as a single or multiple tumors. The development of myeloid sarcoma after allo-SCT is associated with certain cytogenetic abnormalities, developing of graft versus host disease (GVHD), and treatment with donor lymphocytes infusion (DLI). It is believed that posttransplant myeloid sarcomas develop because the EM sites evade immune surveillance. We present two patients with EM myeloid sarcoma in the breast and epipharynx, respectively, as the only manifestation of leukemia relapse. Both patients were treated with a combination of local and systemic therapy, with successfully longtime disease-free survival. Based on these two case reports, we give an updated review of the literature and discuss the pathogenesis, diagnosis, and treatment of EM sarcoma as the only manifestation of AML relapse after allo-SCT. There are no standard guidelines for the treatment of myeloid sarcomas in allotransplant recipients. In our opinion, the treatment of these patients needs to be individualized and should include local treatment (i.e., radiotherapy) combined with systemic therapy (i.e., chemotherapy, immunotherapy, DLI, or retransplantation). The treatment has to consider both the need for sufficient antileukemic efficiency versus the risk of severe complications due to cumulative toxicity

Revmatoid artritt i Norge – demografi, sykdomskarakteristika og behandling. En sammenligning med andre europeiske land og USA

<p><em><strong>Bakgrunn</strong></em>: Revmatoid artritt (RA) er en kronisk inflammatorisk leddsykdom som gir økt sykelighet og dødelighet. Nye biologiske legemidler har de siste 10 årene bedret prognosen betydelig. I 2005 ble QUEST-RA (Quantitative Standard Monitoring of Patients with Rheumatoid Arthritis) prosjektet etablert for å sammenligne sykdomsstatus og behandling hos RA-pasienter i forskjellige land. I denne artikkelen presenteres status for RA-pasienter i Norge sammenlignet med andre europeiske land og USA.</p><p><em><strong>Materiale og metode</strong></em>: Tilfeldig utvalgte RA-pasienter fulgt opp ved revmatologisk poliklinikk ved Sørlandet sykehus i Kristiansand (n=100) og St. Olavs Hospital i Trondheim (n=100) ble inkludert. I henhold til protokoll ble demografiske, sykdoms- og behandlingsdata registrert.</p><p><em><strong>Resultater</strong></em>: Norske RA-pasienter skilte seg lite fra gjennomsnittet i andre land med hensyn til alder, utdannelse og sykdomsvarighet. Sykdomsalvorlighet og sykdomsstatus til norske RA-pasienter er sammenlignbare med pasienter fra land som har lavest sykdomsaktivitet og best helsestatus. I Norge er andelen som behandles med biologiske legemidler ca 30%, og Norge er blant de land med størst andel pasienter som behandles med denne legemiddelgruppen. Fortolkning: RA-pasienter i Norge er blant de i Europa som har lavest sykdomsaktivitet. En årsak antas å være den relativt utbredte bruken av biologiske legemidler i Norge.</p

Hypoglycemia and decreased insulin requirement caused by malignant insulinoma in a type 1 diabetic patient: when the hoof beats are from a zebra, not a horse.

Insulinomas are uncommon tumors, and in patients with diabetes mellitus they are extremely rare. We describe a patient with type 1 diabetes who developed malignant insulinoma. When hypoglycemic episodes persist in a patient with diabetes and treatment‐induced and other causes of hypoglycemia have been ruled out, an insulin‐producing tumor should be considered

Long-Smoldering T-prolymphocytic Leukemia: A Case Report and a Review of the Literature

T-prolymphocytic leukemia (T-PLL) is a rare malignancy of mature T-cells with distinct clinical, cytomorphological, and molecular genetic features. The disease typically presents at an advanced stage, with marked leukocytosis, B symptoms, hepatosplenomegaly, and bone marrow failure. It usually follows an aggressive course from presentation, and the prognosis is often considered dismal; the median overall survival is less than one year with conventional chemotherapy. This case report describes a patient with T-PLL who, after an unusually protracted inactive phase, ultimately progressed to a highly invasive, organ-involving disease. After initial treatments failed, a novel treatment approach resulted in a significant response

Pure Red Cell Aplasia with Del(20q) Sensitive for Immunosuppressive Treatment

Pure red cell aplasia (PRCA) is a rare syndrome that only affects the erythroid lineage. It is defined by a normocytic, normochromic anemia with a marked reticulocytopenia and severe reduction or absence of erythroid precursors in the bone marrow. Treatment of primary, idiopathic PRCA is immunosuppressive therapy. Although it is rare, isolated cytogenetic abnormalities can be seen in PRCA, and abnormal karyotype is associated with poor response to immunosuppressive therapy and poor prognosis. We describe a 77-year-old male with primary, idiopathic PRCA and a deletion of chromosome 20q, del(20q), in the bone marrow cells. He was successfully treated with immunosuppressive therapy and became transfusion-independent. The same cytogenetic abnormality has also been described in a few other reports; taken together, these observations suggest that del(20q) may represent a recurrent cytogenetic abnormality in PRCA. Our case report clearly illustrates that even patients with primary PRCA and an abnormal karyotype can respond to immunosuppression and become transfusion-independent