Search

Objective: Peripheral T-cell lymphomas (PTCLs) are an uncommon and quite heterogeneous group of disorders, representing only 10%-15% of all non-Hodgkin lymphomas. Although both molecular and clinical studies have increased in recent years, we still have little knowledge regarding real-life practice with PTCLs. In this study, we aimed to investigate the clinical characteristics and treatment outcomes of a large population-based cohort of patients presenting with systemic non-cutaneous PTCL. Materials and Methods: We conducted a multicenter retrospective analysis of 190 patients consecutively diagnosed and treated with non-cutaneous PTCLs between 2008 and 2016. Results: Considering all first-line treatment combinations, the overall response rate was 65.9% with 49.4% complete remission (n=81) and 16.5% partial response (n=27). The 5-year overall survival and eventfree survival rates were significantly different between the transplant and non-transplant groups (p, Amaç: Periferik T-hücreli lenfomalar (PTHL) nadir görülen, oldukça heterojen bir grup hastalıktır ve tüm non-Hodgkin lenfomaların sadece %10-15’ini oluşturur. Son yıllarda hem moleküler hem de klinik çalışmalar artmış olsa da PTHL’ler üzerindeki gerçek yaşam verileri hakkında hala çok az bilgiye sahibiz. Bu çalışmada, sistemik, kutanöz olmayan PTHL hastaları içeren geniş popülasyon tabanlı hasta grubunun klinik özellikleri ve tedavi sonuçlarını araştırmayı amaçladık. Gereç ve Yöntemler: 2008 ve 2016 yılları arasında kutanöz olmayan PTHL tanısı ile tedavi edilen 190 ardışık hastanın geriye dönük analizini gerçekleştirdik. Bulgular: Tüm birinci basamak tedavi kombinasyonları dikkate alındığında, genel yanıt oranı; tam remisyon (n=81) %49,4 ve kısmi yanıt (n=27) %16,5 olmak üzere %65,9 saptandı. Beş yıllık genel ve olaysız sağkalım oranları, transplant ve transplant olmayan gruplar arasında önemli ölçüde farklıydı (sırasıyla, p

Although it is well known that numerical and structural chromosomal aberrations are common in leukemic cells, tetraploidy and near-tetraploidy is a rare entity in acute myeloid leukemia (AML) [1-4]. We describe a rare case of AML-M0 with near-tetraploidy and superior vena cava syndrome (SVCS) due to a large mediastinal granulocytic sarcoma (GS). A 25-year-old male was admitted to our hospital with a progressive non-productive cough, dyspnea, pain on the left upper extremity and weight loss. One month ago, he was treated with a diagnosis of venous thrombosis because of the pain and swelling on his left upper extremity. At the time of admittance, physical examination revealed dyspnea, facial edema, swelling of the neck and upper extremities and dilated jugular and superficial veins of the chest. Peripheral lymph node or organomegaly was absent. His hematological findings were: hemoglobin, 14.1 g dl−1; hematocrit, 41.6%; MCV, 87.5 fl; WBC, 15.3 × 109 l−1 with 15% polymorphonuclear cells; 22% bands; 37% lymphocytes; 26% resembling blastic cells; and platelets 284 × 109 l−1. The chest X-ray showed enlarged mediastinum and thorax computed tomography (CT) demonstrated a multi-lobar mass with a diameter of 9 × 5 cm in the anterior mediastinum which also compressed the superior vena cava (Figure 1A). Abdominopelvic CT scan was normal. Mediastinoscopy was performed for definite diagnosis. The biopsy specimens from mediastinal mass revealed diffuse malignant infiltration in lipomatous tissue, which consisted of round, large sized cells. Nuclei were large, vesicular and contained two or three nucleolus. Cytoplasm was moderate in amount and clear. The specimens showed negativity for all of the antibodies and staining except for myeloperoxidase (MPO), CD34 and TdT (Figure 1B). Bone marrow aspirate revealed the presence of large blasts with a relatively low nuclear-cytoplasmic ratio, moderate chromatin pattern, two-to-three prominent nucleoli, no cytoplasmic granules and few cytoplasmic vacuolization. Most of the cells had heterogeneous shaped nuclei. No Auer rod was seen (Figure 1C). Cytochemistry revealed negativity for MPO, Sudan black B, periodic acid Schiff, combined esterase and acid phosphatase. Flow cytometric studies of the bone marrow cells revealed positivity for CD13, CD33, CD34, CD45, CD7 and HLA-DR. Cytogenetic analysis showed near tetraploidy (Figure 1D). Results of cytogenetic analysis of 20 metaphases of the bone marrow cells were as follows; 102, XXYY, +13, +14, +14, +15, +16, +18, +19, +21, +21, +22 [6] / 104, XXYY, +X, +Y, +3, +4, −7, −8, +10, +11, +13, +13, −14, +15, +15, +19, +21, +21, +22, +22 [9] / 98, XXYY, +3, +4, −6, +8, −11, +13, +14, −16, +19, +20, +21, +21 [5]. Neither apparent structural rearrangement nor normal clone was identified. Upon establishing the diagnosis (AML-M0/near tetraploidy+/mediastinal GS+) [5], he was given an induction chemotherapy consisting of daunorubicin (45 mg m−2 per day, days 1–3) and cytarabine (100 mg m−2 per day continuous infusion, days 1–7) and bone marrow remission was achieved. Then, he received the first consolidation chemotherapy consisting of idarubicin (10 mg m−2 per day, days 1–3) and cytarabine (1000 mg m−2 per day, days 1–6), followed by second consolidation chemotherapy consisting of cytarabine (6 gr m−2 per day, days 1–3) and 1800 cGy radiotherapy on mediastinum. The symptoms of SVCS improved gradually after the 5th day of induction chemotherapy. Likewise, the mediastinal mass decreased in size by 30% after induction chemotherapy, 70% after consolidation chemotherapies and disappeared completely after the completion of radiotherapy (Figures 1E and F). Presently, he is in the 10th month of his complete remission without any recurrence in the disease or GS.

Mediterranean spotted fever (MSF), Boutonneuse fever, is a tick-borne disease caused by Rickettsia conorii and is endemic in the Mediterranean region. Although MSF is usually a benign infectious disease, unusual manifestations that have been mainly reported in severe forms, typically among elderly patients or those with underlying diseases, complicate the diagnosis. A 39-year-old woman, a resident of Bursa, Turkey, was admitted with a 5-day history of fever, headache, and malaise and a 3-day history of rash. Physical examination showed fever and maculopapular rash involving the trunk, extremities, palms, and soles. Hematological findings were as follows: Hb 10.8 g/dL, WBC 1.7  109/L with 62% polymorphonuclear cells; 9% bands; 20% lymphocytes; 6% metamyelocytes; 3% myelocytes;and platelet count 46.8  109/L. Laboratory studies revealed an elevated CRP (13 mg/L), accelerated ESR (60 mm/h), hypoalbuminemia (2.5 mg/L),and slightly elevated serum aspartate aminotransferase (53 UI/L) and lactate dehydrogenase (386 UI/L). Bone marrow smear showed hypercellular marrow with activation of myeloid cells. After 3 days of hospitalization, we learned that she has a dog in her yard. A ‘‘tache noire,’’ the typicaleschar at the site of the tick bite, was observed on the right inguinal region with more elaborate physical examination. Treatment with doxycycline (200mg/day) was started. Resolution of fever and normalization of haematological findings were observed on the 4th and the 6th days of treatment, respectively.

We read with great interest the article by Disel and colleagues [1] in a recent issue of Leukemia Research that described a case of acute myelogeneous leukemia (AML) with bilateral ear neutrophil poor Sweet's syndrome (SS). We would like to describe and share our experience in a patient with AML who developed bilateral eyelid SS and add some points concerning the SS. Sweet's syndrome, also referred to as febrile neutrophilic dermatosis, is characterized by fever, neutrophilic leukocytosis, painful red papules, nodules and plaques that respond readily to corticosteroid therapy [2], [3]. Although SS has been linked to hematologic malignancies, the frequency of SS with leukaemia is even lower in patients with neutropenia [4].

We read with great interest the article by Ault et al1 in a recent issue of Journal of Clinical Oncology, describing the results of pregnancy among patients with chronic myeloid leukemia (CML) treated with imatinib. We would like to add some points and bring attention to some questions concerning imatinib and pregnancy. Tyrosine kinases (TKs) are one of the most critical groups of signaling molecules for the cellular regulation of proliferation, differentiation, survival, function, and motility, and various tumors overexpress TKs, leading to uncontrolled mitogenic signals to the neoplastic cells. Imatinib (Gleevec or Glivec; Novartis, Basel, Switzerland) is a small-molecule tyrosine kinase inhibitor active against BCR-ABL, c-ABL, ARG, PDGF-r, and c-KIT.2 Although imatinib has been used primarily for cancers such as CML, use of imatinib has demonstrated that tyrosine kinase inhibitors can have a wide therapeutic window and heralds the era of targeted cancer and noncancer disease therapy. Information about the effects of drugs on the developing embryo is derived primarily from animal experiments and case reports describing the outcomes of pregnancies complicated with drug. Some drugs can be teratogenic in some animal species, but not in humans. In preclinical studies imatinib was found to be teratogenic in rats, but not in rabbits, and impaired spermatogenesis was observed in rats, dogs, and monkeys; however, there was no evidence that imatinib was genotoxic. These observations lead to concerns that men treated with imatinib may have reduced sperm counts. Clinical experience has not shown this to be true because male patients who were receiving treatment of imatinib were partners in 18 pregnancies and four healthy infants. Owing to teratogenicity data in rats (causing exencephaly or encephalocele, and absent or reduced frontal and absent parietal bones), it is recommended that women treated with imatinib be aware of the potential teratogenicity of imatinib, and effective contraception should be used during imatinib therapy to prevent pregnancy.2 Hensley and Ford2 reported 26 pregnancies (15 in clinical trials, 11 in nonclinical trials) among women taking imatinib. Most of the patients opted for elective therapeutic abortions, and only three patients carried their pregnancies to term. Two infants were healthy, and one infant had hypospadias. Recently, eight patients with CML who became pregnant while receiving imatinib therapy were reported from different areas of the world.3-7 All of the fetuses had been exposed to imatinib during the first trimester, and four fetuses have also been exposed to imatinib during the second and third trimesters. Six of the reported eight pregnancies proceeded to term with healthy infants, and two were terminated with unsatisfactory outcomes—one had spontaneous abortion4 and the other had a dead fetus with meningocele.7 More recently, Ault et al1 reported their experience on 19 pregnancies involving 18 patients (10 female and eight male patients) who conceived while receiving imatinib for the treatment of CML. Three pregnancies (involving two female patients and one male patient) ended in spontaneous abortion, and one patient had an elective abortion. All other pregnancies were uneventful. Two of 16 infants had minor abnormalities at or shortly after birth (hypospadias in one and rotation of small intestine in another) that were surgically repaired.