Supportive care in severe and very severe aplastic anemia.

Optimal management of aplastic anemia (AA) is not confined to immediate diagnosis, early decision making and timely initiation of major treatment strategies (immunosuppression or SCT) but also involves supportive treatment as a crucial part of patient care. Patients are threatened by complications of cytopenia. Here, we summarize current recommendations for prevention and early treatment of fungal, bacterial and viral infections, transfusion strategy and iron chelation and assess the evidence basis. In fact, many recommendations for patients with AA are not based on randomized studies in AA itself, but they are deduced from other conditions with similar severity of cytopenia. Prevention and treatment of complications like hemorrhage, bacterial and fungal infections and of secondary events like alloimmunization to blood products and iron overload have a significant impact on the prognosis of AA patients and need to be carefully observed in daily practice. More controlled studies on supportive care should be performed in this rare disease.

Total body irradiation prior to bone marrow transplantation--the experience of the Institute of Oncology Prof. Dr. Al. Trestioreanu Bucharest.

PURPOSE: To present the technique of total body irradiation (TBI), applied for the first time in Romania, at the Institute of Oncology Bucharest, as part of stem cell transplantation for hematological malignancies. PATIENTS AND METHODS: The total dose administered was 12 Gy at the reference point, 2 Gy/fraction, one fraction per day, 6 consecutive days, with a total dose of 8 - 11.4 Gy delivered to the lung, using Mevatron Primus linear accelerator (6 MV & 15 MV, 200-300 cGy/min in isocenter), in vivo dosimetry detectors and equipment for the reference dosimetry, personalized blocks for lung shielding sustained by polymethylmethaacrylate (PPMA) plate, Simulix HP simulator, and computer tomographic (CT) scans. Techniques used were: a) two parallel opposed anteroposterior / posteroanterior (AP/PA) fields with the patient in prone and supine position; b) two parallel opposed lateral fields with the patient placed on a lateral table, at 320 cm from the source. The percentage depth dose, tissue maximum ratio (TMR), off axis ratio (OAR) and the reference dose rate were measured for every patient's geometrical characteristics, with an uncertainty of +/- 2.2% and were used to calculate monitor units and to evaluate the dose in organs at risk (lungs, gonads, eyes etc). RESULTS: 5 patients (3 with the AP/PA technique and 2 with the lateral technique) were irradiated. All patients completed their irradiation in good clinical condition. The acute side effects were minimal (WHO grade 1: nausea/ vomiting--all patients; diarrhea--1 patient; headache--2 patients; photophobia and diplopia--1 patient; head and neck skin erythema--all patients). Because of the short follow-up period no safe evaluation of late side effects can be done. However, during this period one patient developed a non-aggressive form of chronic liver graft vs. host disease (GVHD) and one patient died due to acute GVHD. CONCLUSION: TBI as part of stem cell transplantation for hematological malignancies was successfully realized at our Institute, with favorable clinical results. This technique is easy to carry out and reproducible.

[Laparoscopic subtotal splenectomy in the treatment of hereditary spherocytosis]. / Splenectomia subtotala laparoscopica în tratamentul microsferocitozei ereditare.

Clinical manifestations of hereditary spherocytosis, the most common red blood cell membrane disorder, can be controlled by splenectomy. However, concerns regarding exposure of patients to a life long risk for overwhelming infections have restricted its use, especially în children. Subtotal splenectomy, as long as 80% to 90% of the enlarged spleen is removed, is a logical alternative. Subtotal splenectomy was effective în decreasing the hemolytic rate, while maintaining the phagocytic and immune function of the spleen. This surgical procedure should be considered în transfusion-dependent infants and children whit hereditary spherocytosis and în older patients whit erythrocyte membrane defects. Based on our experience în laparoscopy (120 laparoscopic splenectomies) and open subtotal splenectomy (5 cases) we performed 2 laparoscopic subtotal splenectomies in patients with hereditary microspherocytosis with good short term results. We have had no problem with blood loss and no transfusions were needed. The procedure can be performed safely and easily with all the traditional advantages of a minimally invasive approach. In order to evaluate the long term clinical benefit a minimal follow-up of 5 years is needed.

[A case of disseminated intravascular coagulation--a paraneoplastic manifestation of a borderline ovarian tumor]. / Consideratii asupra unui caz de coagulare intravasculara diseminata (CID)--manifestare paraneoplazica a unei tumori ovariene de granita.

A 24-year-old-woman was admitted because of disseminated intravascular coagulation (DIC), menometrorrhagia and galactorrhea. The investigations performed showed a right adnexal tumor after the equilibration of DIC with plasma substitution, we performed a right adnexectomy with limited excision of peritoneal. The pathologic examination showed a focus of endometriosis on the right ovary who had a polycystic look and a right adnexal fibromyxoma (premalignant lesion). The post operative evolution was good, with the loss of entire onco-hemato-endocrinologic picture. We describe the interrelation between DIC (paraneoplastic syndrome), menometrorrhagia and galactorrhea, the pathologic hypothesis and the treatment of DIC.

The value of the ultrastructural pattern of myeloperoxidase (MPO), platelet peroxidase (PPO) and acid phosphatase in identification of blastic cell types during acute transformation of chronic myeloproliferative disorders (cMPD).

We established the cell line of bone marrow and blood cells provided by 19 cases of cMPD by ultrastructural studies of their enzymatic content. Myeloblasts, megakaryoblasts and lymphoblasts were identified by the presence of myeloperoxidase, platelet peroxidase and acid phosphatase, respectively.

The value of the ultrastructural pattern of myeloperoxidase and platelet peroxidase in identification of two blastic cell types during transformation of a chronic myeloproliferative disorder.

The value of the ultrastructural pattern of myeloperoxidase (MPO) and plateletperoxidase (PPO) staining is evaluated for the identification of the type of blastic cells during the acute transformation of a chronic myeloproliferative disorder. MPO and PPO are generally accepted as lineage specific markers for the detection of myeloid or megakaryocytic differentiation of the blast cells. The ultrastructural pattern of myeloperoxidase (MPO) and plateletperoxidase (PPO) permitted us to identify two types of blastic cells (one from the myeloid and the other from the megakaryoblastic lineage) in the acute transformation of a chronic myeloproliferative disorders.

Ultrastructural aspects of megakaryocyte/platelet system in essential thrombocythemia.

Six cases of essential thrombocythemia (ET) have been investigated in bone marrow as well as in blood cells. In majority of the cases, the bone marrow aspirate was hypercellular and presented an increased number of megakaryocytes, some of them with displastic appearance. The ultrastructural pattern of platelet peroxidase permitted us to identify atypic megakaryocytes and megakaryoblasts, particularly when present in pheripheral blood.