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.
Key Clinical MessageInsulinomas 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.
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Although rare, acute myelogenous leukemia (AML) can include extramedullary manifestations, sometimes presenting as a solid tumor called a myeloid sarcoma. Myeloid sarcoma can be the cause of the initial presenting complaint before AML diagnosis, or may be detected as a sign of disease-relapse after treatment. Here, we report a case in which the initial presentation included abdominal discomfort and signs of kidney failure. Further investigation revealed signs of unilateral hydronephrosis. Due to a diagnostic delay, the patient was diagnosed with AML with extramedullary manifestation only after the development of full-blown leukemia. Biopsy of the compressive tumor confirmed an extramedullary myeloid sarcoma, and [18F]-FDG-PET/CT proved useful for patient diagnosis and follow-up. This case report illustrates the importance of thorough examination and diagnosis, as a serious underlying disease with a rare cause can debut with an unusual presentation.
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.
En kvinne i 40-årene med bekkensmerter og uklare MR-funn | Tidsskrift for Den norske legeforening En kvinne i 40-årene med bekkensmerter og uklare MR-funn NOE Å LAERE AV MARTE KAREN BRATTÅS Medisinsk avdeling Haraldsplass Diakonale Sykehus Marte Karen Brattås er lege i spesialisering i indremedisin. Forfatteren har fylt ut ICMJE-skjemaet og oppgir ingen interessekonflikter.
Background The QUEST-RA project (Quantitative Standard Monitoring of Patients with Rheumatoid Arthritis) a multi centre and multinational project has revealed significant differences in disease severity and treatment in RA patients in European countries (1,2). Norway joined QUEST-RA in 2009. Objectives To compare Norwegian RA patients with other European countries using the QUEST-RA protocol. Methods From two ordinary rheumatology outpatient clinics in Norway (Kristiansand and Trondheim) 200 consecutive RA patients 100 from each centre using the QUEST-RA protocol was included. Data on demographics, disease severity and treatment was collected. Data collection was finished spring 2010. Continuous variables are presented as mean (SD) or median [range]. Results Demographic: age 58 (13) years, females 65.5%, BMI 25.7 kg/m2, Caucasian 98.5%, current smoker 26.6%, education 11.0 [4-20] years, full time employed 17.1%, full time employed age <65 years 25.0%, disease duration 13.3 (10.9) years. Disease activity: DAS28 3.6 (1.4), 28 joint count swollen 1.0 [0-21] and tender 3.0 [0-28], doctors global 1.8 [0-7.7] mm, ESR 14 [2-78] mm/hr. Patient reported outcomes measures (PROMs): HAQ 0.88 [0-2.63], pain 3.5 [0-9.3] cm, fatigue 3.8 [0-10] cm, patient global 3.8 [0-9.8] cm, morning stiffness 45.0 [0-301] min. Disease markers: RF+ 73.1%, erosive status 61.5%. Treatment: biologics 27.0%, methotrexate 59.5%, other DMARDs 14.0%, prednisolone 52.0%. Significant (p<0.05) gender differences (men/women) were found for the following variables: full time employed <65 years (47.7%/14.1%), DAS28 (3.1/3.9), HAQ (0.50/0.88). No significant gender differences were found for e.g. swollen joints, ESR, RF and erosions and treatment. In comparison with other European QUEST-RA countries disease activity and health status in Norwegian RA patients were comparable with countries that had reported the lowest disease activity (DAS28) and the best health status in their RA patients (see table below). The proportion of RA patients treated with biologics in Norway is among the highest in Europe. DAS28 (mean)HAQ (median)Pain (median)ESR (median)Biologics (%) Norway3.60.883.51427 Sweden3.80.883.31927 Holland3.10.752.51520 Finland3.30.632.81313 Germany4.40.755.02023 Italy4.51.004.92813 Spain3.50.883.11723 Hungary5.11.385.22612 Polen5.31.385.0316 Conclusions Norway is among European countries reporting the lowest level of disease activity in their RA patients and is also among countries with the highest proportion of patients on biologics. This however cannot be explained by use of biologics alone as e.g. Finland (1) using significant less biologics than Norway is reporting even a lower disease activity in their RA patients. References Sokka T et al. Ann Rheum Dis. 2007;66:1491-6. Sokka T et al. Ann Rheum Dis. 2009;68:1666-72. Disclosure of Interest G. Haugeberg Grant/Research support from: Abbott Norway, E. Wilberg: None Declared, H. Gjelberg: None Declared, E. Rødevand: None Declared, T. Isler Grant/Research support from: Abbott International
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