Ülkü Ozan

The bone marrow aspirate and biopsy in the diagnosis of unsuspected nonhematologic malignancy: a clinical study of 19 cases.

BackgroundAlthough bone marrow metastases can be found commonly in some malignant tumors, diagnosing a nonhematologic malignancy from marrow is not a usual event.MethodsTo underscore the value of bone marrow aspiration and biopsy as a short cut in establishing a diagnosis for disseminated tumors, we reviewed 19 patients with nonhematologic malignancies who initially had diagnosis from bone marrow.ResultsThe main indications for bone marrow examination were microangiopathic hemolytic anemia (MAHA), leukoerythroblastosis (LEB) and unexplained cytopenias. Bone marrow aspiration was not diagnostic due to dry tap or inadequate material in 6 cases. Biopsy results were parallel to the cytological ones in all cases except one; however a meticulous second examination of the biopsy confirmed the cytologic diagnosis in this patient too. The most common histologic subtype was adenocarcinoma, and after all the clinical and laboratory evaluations, the primary focus was disclosed definitively in ten patients (5 stomach, 3 prostate, 1 lung, 1 muscle) and probably in four patients (3 gastrointestinal tract, 1 lung). All work up failed in five patients and these cases were classified as tumor of unknown origin (TUO).ConclusionOur series showed that anemia, thrombocytopenia, elevated red cell distribution width (RDW) and hypoproteinemia formed a uniform tetrad in patients with disseminated tumors that were diagnosed via bone marrow examination. The prognosis of patients was very poor and survivals were only a few days or weeks (except for 4 patients whose survivals were longer). We concluded that MAHA, LEB and unexplained cytopenias are strong indicators of the necessity of bone marrow examination. Because of the very short survival of many patients, all investigational procedures should be judged in view of their rationality, and should be focused on treatable primary tumors.

Maternal and fetal outcomes in pregnancy complicated with acute leukemia: a single institutional experience with 10 pregnancies at 16 years

The incidence of acute leukemia in pregnancy is low and the management of acute leukemia during pregnancy is difficult. We have observed a total of 10 pregnancies in 8 patients. Six of the patients had acute myeloblastic leukemia (AML) and two of them had acute lymphoblastic leukemia (ALL). Three of the pregnancies were diagnosed when the leukemia was in remission, six at the time of leukemia diagnosis and one at the time of leukemic relapse. Six of the pregnancies were found in first trimester, three in the second and one early in the third. Three pregnancies ended with spontaneous abortion, three with intrauterine death and three with medical termination. One of spontaneous abortions and one intrauterine death developed during combination chemotherapy (daunorubicin, cytarabine). Only 1 healthy baby survived from the 10 pregnancies and this child was the not exposed to chemotherapeutic agents. None of the cases had gynecologic and obstetric complications. Five of eight pregnant women with leukemia died because of the primary disease.

Idiopathic thrombocytopenic purpura in pregnancy: a single institutional experience with maternal and neonatal outcomes

We observed 13 pregnant women of 70 females with idiopathic thrombocytopenic purpura (ITP) from January 1992 through September 2002. Thirteen mothers with ITP gave birth to twelve babies and two fetuses died. One of the pregnancies produced twins. Seven of the cases were diagnosed with ITP before pregnancy and six during pregnancy. One of the thirteen pregnancies was complicated by preeclampsia, one by ablatio placentae, and one by intrauterine death. Seven mothers received corticosteroid treatment, four high-dose immunoglobulin therapies, and one underwent splenectomy in the second trimester of gestation. At the time of delivery six mothers had normal platelet counts and seven had low platelet counts. Nine deliveries were by vaginal route and four were by cesarean section. Eleven infants were born with normal platelet counts and one was thrombocytopenic at the time of delivery. No infant showed any clinical signs of hemorrhage and there were no neonatal complications. Two fetuses died; one of them because of ablatio placentae and the other was intrauterine dead. In conclusion, ITP in pregnancy requires the management of two patients, the mother and her baby; hence, the close collaboration of a multidisciplinary group composed of a hematologist, obstetrician, anesthesiologist, and neonatologist is essential.

Rhabdomyosarcoma of the perianal region presenting as acute leukemia

Rhabdomyosarcoma (RMS) is the most common malignant soft tissue tumor in adolescence and childhood, which becomes manifest by the locally destructive growth of the primary tumor or its metastases. Sometimes no discernible primary lesion can be detected despite widespread dissemination or in some rare cases the disease may be confined to the marrow. Although approximately 60% of soft tissue sarcomas of children are RMS, it constitutes only 2–5% of all soft tissue sarcomas in adults [1–3]. A 25-year-old female presented with pain and mass of the perianal region, fatigue, fever, and weight loss. Her history revealed that she had developed a papular lesion in her perianal region 4 months before her admittance, which enlarged and acquired a large mass with time. Concomitantly she had symptoms of anemia and weight loss. One month before her admittance, she underwent drainage of the mass as an abscess and had five erythrocyte transfusions at that time. Two weeks before admission, she developed dizziness, epistaxis, and 38°C fever and was referred to our unit with a diagnosis of acute leukemia. Physical examination revealed severe pallor, petechiae on the lower extremities, lymph node with dimension 2×2 cm in the right inguinal region, and a hyperemic, ulcerated, and infected mass with dimensions of 15×10 cm in the lower right gluteus. Her hematologic findings were: hemoglobin 5.3 g/dl, hematocrit 15.3%, mean corpuscular volume (MCV) 80 fl, mean corpuscular hemoglobin (MCHb) 28 pg, WBC 7.69×10/l with 53% polymorphonuclear cells, 16% bands, 18% lymphocytes, 9% monocytes, and 4% blasts, and platelets 107×10/l. Bone marrow smear showed that 90% of marrow cells were tumor cells. They were characterized by vacuolated cytoplasm and the propensity to form pairs, clusters, and multinucleated forms (Fig. 1a–c). Flow cytometric studies of the bone marrow cells revealed no lineage markers for B cells, T cells, or myeloid cells. Trephine biopsy showed complete replacement of marrow (Fig. 1d) and infiltration of the mass by small round cells (Fig. 1e), which stained positively for actin, desmin, and vimentin, but negatively for leukocyte common antigen (LCA) and cytokeratin. The diagnosis of alveolar rhabdomyosarcoma (ARMS) was established. Abdominopelvic computed tomography showed a mass with dimensions of 14×12×10 cm in the medial region of right gluteus and the other masses with 4×3 cm near the inferior ramus pubis and in the anterior part of the right piriform muscle (Fig. 1f). The patient underwent a combined regimen of chemotherapy. Initially the neoplasm responded to chemotherapy, but disease progressed after 4 months. The histological diagnosis of tumor often remains difficult in a variety of solid tumors characterized by round cell morphology including RMS, neuroblastoma, Ewing’s sarcoma, and some cases of non-Hodgkin’s lymphoma [3]. Relatively few conditions spuriously may mimic a hematological disease such as acute leukemia; one of these is ARMS. ARMS in cases of bone marrow involvement is easily confused with acute leukemia and also the clinical picture may mimic the similar systemic symptoms of acute leukemia. The cells of ARMS resemble hematologic blasts (particularly lymphoblasts) and are difficult to differentiate from leukemic cells [2, 4]. R. Ali (*) . F. Özkalemkaş . Ü. Ozan . T. Özçelik . V. Özkocaman . A. Tunalı Department of Internal Medicine, Division of Hematology, Uludağ University School of Medicine, Bursa, Turkey e-mail: ridvanali@uludag.edu.tr Tel.: +90-224-4428400 Fax: +90-224-4428060

Successful treatment of acquired haemophilia with prednisolone therapy

Summary.  Acquired hemophilia is a rare, life threatening coagulopathy in adults caused by the development of autoantibodies against to factor VIII. No general consensus exists on the best therapeutic approach. We report here a case that presented with extensive cutaneous and mucosal bleedings due to factor VIII inhibitors and treated successfully with steroid therapy alone but complicated with a life threatening thromboembolic attack during her follow up. In conclusion, corticosteroids are “cost effective therapy” associated with high inhibitor elimination rates and although recurrence of inhibitor in a patient with factor VIII inhibitor is an expected clinical situation thrombosis risk should also be considered.

Successful treatment of BALT lymphoma with combined chemotherapy

Primary pulmonary lymphoma arising from mucosa- or bronchus-associated lymphoid tissue (MALT or BALT) is a rare disease.1,2 We report a 30 year old man with low grade B cell BALT lymphoma who presented with a bulky mass in the lung and was treated successfully with cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP) combined chemotherapy. The patient was admitted with a dry cough, shortness of breath, back pain, and progressive infiltrates on chest radiographs. He had no …

Primary Non-Hodgkin's T-cell Lymphoma of Bone

A 38-year-old male presented with pain and a mass in the right hypochondrium. A CT scan of the thorax showed a bulky mass with a diameter of 106 10 cm at 11th right rib. Surgical resection yielded the histological diagnosis of a diffuse large T-cell lymphoma. No lymphadenopathy or organomegaly or infiltration of bone marrow was detected. The patient underwent combined chemotherapy CHOP (Cyclophosphamide 750 mg/m i.v. day 1, Doxorubicin 50 mg/m i.v. day 1, Vincristine 2 mg i.v. day 1, Prednisone 100 mg p.o. days 1 – 5) which was repeated every 3 weeks. After the second CHOP treatment the patient complained pain in the left proximal femur. Conventional X-ray showed lytic lesions in the left ramus pubis (Fig. 1). MR showed a mass that was arising from left ramus pubis and infiltrating the obturatorius, pectineus, levator muscles and destroying the posterior tuber ischiadicum (Fig. 2). The patient received involved-field radiotherapy (RT). During RT the patient complained of extensive pain accompanied by progressive weakness. A bone scan revealed uptake of technetium at multiple sites (right proximal humerus, left proximal clavicula, right 9th rib, left 10th rib, left sacroiliac joint, left acetabulum and left ischion) (Fig. 3). Completing two cycles of CHOP treatment and radiotherapy the patient underwent salvage chemotherapy ESHAP (Etoposide 40 mg/m i.v. (1 h) days 1 – 4, Methylprednisolone 500 mg/m i.v. (15 min) days 1 – 5, Cytarabine 2 gr/m i.v. (2 h) day 5, Cisplatin 25 mg/m i.v. (continuous infusion) days 1 – 4) which was repeated every 3 weeks, but after the third ESHAP therapy he died without achieving a partial or complete response.