INTRODUCTION: Hereditary thrombophilia is caused by various inherited disorders which lead to familial tendency to recurrent venous thrombosis usually at an early age and with spontaneous onset. In the studies reported so far, the different prevalence of hereditary thrombophilia among patients with venous thrombosis was found, greatly depending on criteria for selection of patients. Arterial thrombosis is most often the consequence of arteriosclerosis but the prevalence of hereditary thrombophilia among young patients with arterial thrombosis and without recognized risk factors for arteriosclerosis is not known . In this study, the frequency of hereditary deficiencies of antithrombin III (AT III), protein C (PC), protein S (PS), plasminogen (PLMG), factor XII (F XII) and dysfibrinogenaemia was investigated over a 2-year period in 121 patients with venous or arterial thrombosis selected according to the recommendations of the British Committee for Standards in Haematology. PATIENTS AND METHODS: The study included total a of 121 patients (58 males and 63 females) with documented venous or arterial thrombosis. Table 1 shows patient's characteristics regarding gender, age and clinical manifestation of thrombosis. Each patient fulfilled at least one of the following criteria: a) venous thrombosis prior to the age of 45; b) arterial thrombosis prior to the age of 30, without risk factors for arteriosclerosis; c) recurrent thrombosis; d) familial tendency to thrombosis; e) thrombosis of unusual localization. A detailed history was taken from each patient on earlier personal or familial occurrence of thrombosis. For the purpose of this study, thrombophilia was characterized as congenital when the deficient protein was constantly below normal value and when the same deficiency was confirmed in a close family member; acquired when the acquired disorder predisposing to thrombosis was present in absence of constant protein deficiency; and idiopathic when the cause of thrombosis was unknown. All tests were performed in plasma obtained after centrifugation of venous blood anticoagulated with 0.129 mol/1 sodium citrate. Concentrations of fibrinogen, PT, PTT and F XII were measured by standard clotting methods. At III, PC and plasminogen activity were determined by chromogenic methods using commercial reagents (Boehring, Marburg, Germany). AT III, PC and total PS antigen were assayed by Laurell immunoelectrophoresis. The presence of lupus anticoagulant was investigated by recommended tests. RESULTS: A total of 15 patients (12.4%) fulfilled criteria for hereditary thrombophilia. Seven of them (5.8%) had AT III deficiency, five (4.1%) PC deficiency, two (1.6%) PS deficiency, and one patient had F XII deficiency. Secondary thrombophilia was found in 21.5% of patients and the cause of thrombosis in 66.1% of patients was not elucidated. A high frequency of hereditary thrombophilia has been found in patients with arterial thrombosis (40%). Among patients with hereditary thrombophilia thrombosis occurred at significantly younger age (29.9 vs. 42.2 and 40.9 yr.) compared to the patients with secondary and idiopathic thrombophilia, respectively. Patients with hereditary thrombophilia had also a higher occurrence of positive family history related to thrombosis (66.7% vs. 7.7% and 27.5%). DISCUSSION: The prevalence of hereditary thrombophilia in nonselected patients with venous thrombosis is relatively low, and for that reason the selection of patients, according recommended criteria, in whom the screening tests for congenital thrombophilia should be performed, is strongly suggested by many authors. In our study we used the generally accepted recommendations for investigation of patients with venous and arterial thrombosis. The presence of congenital thrombophilia was found in 15 (12.4%) of 121 studied patients, what is in accordance with results of other similarly designed studies. (ABSTRACT TRUNCATED)

Adolescent↗

PubMedDate not supplied

[Angioblastic lymphadenopathy--its course and prognosis].

INTRODUCTION: In recent years important advances have been made in the understanding of angioimmunoblastic lymphadenopathy since substantial controversy has been related to the name, course, prognosis and therapy of the disease. It was first recognized in the Kil Classification as a low risk T-cell lymphoma [5], and omitted from the most widely used Working Formulation for clinical purposes. According to the criteria of REAL (Revised European American Lymphoma), classification angioimmunoblastic lymphadenopathy (AILD) is one of peripheral postthymic T cell lymphomas that are an immunologically defined category of non-Hodgkin's lymphomas originating from the peripheral lymphatic tissues. Morphologically, AILD is characterized by partially or completely obliterated sinuses and frequent infiltration of the pericapsular tissue and substantial proliferation of epithelioid, postcapillary venules. Cytologically, polymorphous cellular infiltration with immunoblasts, transformed lymphoid cells, polyclonal plasma cells, eosinophils and epithelioid cells are found. Clinically, rapid occurrence of systemic symptoms in elderly individuals (sixth and seventh decades of life) with generalized lymphadenopathy, hepatosplenomegaly and cutaneous maculo-papulous or erythematous rash is noted. The patients are characterized with hyperimmune condition in the form of Coombs' positive haemolytic anaemia, polyclonal hypergamma-globulinaemia and liability to infections [8, 9]. In spite of numerous suggestions, therapeutic consensus has not been achieved, and the reported survival ranges from 1 to 30 months [10, 11]. Therefore, this information suggests an aggressive form of the disease with the 60% mortality rate. METHODS: At the Institute of Haematology of the Clinical Centre of Serbia in Belgrade in the last five years, from 1993 through August 1998, nine patients were diagnosed with AILD according to the results of pathohistological examination of the extirpated peripheral lymph nodes and the correlation with clinical picture and relevant laboratory findings. RESULTS: Clinical characteristics of nine patients in whom AILD was diagnosed after lymph node biopsy are given in Table 1. The group consisted of 6 men and 3 women, mean age 53. Eight patients were in advanced stage of the disease at the time of the diagnosis (III and IC CS), while the patient in II CS stage had a large tumorous mass (M+). All patients had initial systemic symptoms. Five of them developed fever with chills. Three patients had evidence of extranodal infiltration of the bone marrow. Infiltration of the liver was suspected in two patients according to aberrant hepatogram values, although pathohistological verification was not obtained. In one patient lung infiltration was histologically verified in addition to bone marrow and liver infiltration. All patients had peripheral lymphadenopathy, and most of them hepatosplenomegaly, as well. Three patients had the so called bulky form of the disease since the diameter of the largest tumour exceeded 10 cm. On admission, most were in poor overall condition, and only two were apparently healthy. Knowing that AILD is basically an immunoregulatory disease and that the described cases of association with systemic diseases of the connective tissue and some drugs were implied in the triggering of AILD, Table 2 shows important information obtained form histories of these patients. Namely, 7 of 9 patients had cutaneous changes suggestive of erythematous or maculopapular rash, while three had received corticosteroid therapy for months before AILD was diagnosed since toxoallergic exanthema had been incorrectly suspected. Three patients received gold sodium thiosulfate therapy for rheumatoid arthritis, while four had history of allergy to drugs and pollen. Table 3 shows laboratory results: anaemia was present in 8 of 9 patients, it was severe in three with haemoglobin values of 67 g/L, 72 g/L and 50 g/L, respectively. Five patients had haemolysis. A

Adult↗

PubMedDate not supplied

[T-cell-rich B-cell lymphoma].

The authors present three patients, one female and two males, with special forms of non-Hodgkin's lymphoma, the so called T-cell rich B-cell lymphoma (TCRBCL). This is a relatively new entity which has not yet been described in our literature. The first diagnosis in our female patient was Hodgkin's disease. However, the revision of old and new lymph node samples and use of immunohistochemical analysis helped the establishing of diagnosis--TCRBCL. In the other two patients the diagnosis of TCRBCL was established on the basis of immunohistochemical analysis of the lymph node which indicated the dominating T-lymphocytes, positive to pan-T antigen (CD45 RO+), with considerably lower count of B-lymphocyte elements positive to CD20+. The female patient survived 33 months from the time of diagnosis. The other two patients experienced complete remission lasting 10 and 12 months after the end of therapy.

Adult↗

PubMedDate not supplied

[Histopathologic characteristics of bone marrow in patients with aplastic anemia].

INTRODUCTION: Histopathologic findings in bone marrow biopsies in patients are with aplastic anaemia crucial for the diagnosis of the disease. Changes observed by the examination of bone marrow such as cellularity, histopathologic type, presence of irregular cells maturation in all three blood lineages, condition of medullar stroma can be of great prognostic significance [1, 2, 4, 5]. The aim of the study was to make histopathological analyses in patients with aplastic anaemia and to establish parameters significant for the prognosis of the disease. PATIENTS AND METHODS: Thirty-three patients with aplastic anaemia treated in the Institute of Haematology, Clinical Centre of Serbia, from 1988 to 1995 were included in the study. Bone marrow specimens were analysed for: cellularity, number of megakaryocytes, condition of medullar stroma, presence/absence of blood and oedema, lymphoid elements, presence of plasma and reticular cells, and determination of histopathologic type on the basis of bone marrow changes. For data description we used the arithmetic mean and standard deviation for parametric results. Results of analyses were explained by Kaplan-Mayer's method and two factors analysis of variance. RESULTS: In all analysed marrow biopsy specimens a 100% hypocellularity was observed: 1. Weak hypocellularity in two specimens (6%); 2. Mild hypocellularity in six cases (19%); 3. Severe hypocellularity in 24 (75%), In one case (3%) the finding was not known. In 25 specimens (75%) megakaryocytes were not found, in 5 (16%) they were rare; in two cases (6%) they were numerous. Medullar stroma changes were not found in 30 (94%) byopsies, but in two (6%) they were found. Blood and oedema liquid were found in 26 (81%) cases and not in 6 (19%). Lympho-plasmocytic bone marrow infiltration was found in 23 specimens (72%) and not in nine (28%). First histopathologic types two (one-a and one-b) were present in 25 (78%) cases, histopathologic type two in 5 (16%) and type three in 2 (6%) specimens. The two factors analysis of variance regarding proportions between dead and alive patients revealed a significant difference in proportions according to histopathologic types (FD = 7.52; DF = 2; p = 0.002). Significant statistical differences were found between alive and dead patients in all three histopathologic types, tip one (one-a and one-b) (FD = 8; DF = 1; p = 0.06); type two (FD = 4.286; DF = 1; p = 0.041) and type three (FD = 4.762; DF = 1; p = 0.031). Histopathologic type two had the best prognosis regarding survival probability; p = 0.800; in histopathologic type two survival prognosis was worse p = 0.300, and type three it was the worst, p = 0.000. With regard to survival time, there was no statistical difference in patients with weak and mild bone marrow hypocellularity (p = 0.487). We also compared patients with severe bone marrow hypocellularity with patients with weak and mild hypocellularity. The difference in survival was statistically significant (p = 0.026), Consequently, survival was shorter in patients with severe bone marrow hypocellularity. DISCUSSION: Bone marrow biopsy and histopathologic examination are necessary for definitive diagnosis of aplastic anaemia [1-3]. Cellularity estimation in bone marrow based on biopsy and histopathologic examination is more reliable than that made by bone marrow aspiration [7]. Aplastic anaemia is a disease that can be associated with abnormal clones development. Most frequently paroxysmal nocturnal haemoglobinuria, acute leukaemias and myelodysplastic syndrome occur. Therefore, repeated bone marrow biopsies are necessary to follow-up the course of the disease [10, 11]. The presence of megakaryocytes in bone marrow specimens has favourable prognostic significance. Their appearance is a reliable sign of disease improvement [6, 7]. However, lymphocytosis, plasmocytosis and damaged marrow stroma are markers of bad prognosis [7]. (ABSTRACT TRUNCATED)

Analysis of Variance↗

PubMedDate not supplied

[Extramedullary blast transformation in chronic granulocytic leukemia].

A patient with Ph1(Philadelphia chromosome) positive chronic granulocytic leukemia and extramedullary blast transformation (crisis) in lymph nodes of neck and axillae which appeared after 4-year of treatment with busulfan is presented. Biopsy of lymph node and histopathological examination showed lymphoblastic infiltration. The patient was treated with radiotherapy and chemotherapy with protocol COP. He survived 7 months and expired due to renal insufficiency.

Blast Crisis↗

PubMedDate not supplied

[Orbital lymphomas].

Lymphomas are relatively rare in the orbit: only about 8-11% of all orbital tumours. The most common histological type is diffuse well-differentiated lymphocytic lymphoma (DWDLL--Rappaport's classification)--70%. Orbital lesions are always curable by radiotherapy, but in some 40% of these patients local recurrence or dissemination occur. The authors present two patients with diffuse well-differentiated lymphocytic lymphoma, and one patient with FCC lymphoma of centrocytic type (Kiel classification) localized in the orbit. Two of them were irradiated after an unsuccessful treatment by chemotherapy. In the third patient local radiotherapy is still in the course.

Aged↗

PubMedDate not supplied

[Acute lymphoblastic leukemia in refractory anemia with "ringed" sideroblasts].

The paper deals with the progression of idiopathic refractory sideroblastic anaemia (IRSA) and its transformation to acute B lymphoblastic leukaemia (ALL). Attention is paid to haematological changes prior to leukaemia development. Acute leukaemia was best expressed in this patient by severe deterioration of dyserythropoiesis, leukopenia, and by an increase of blasts in the bone marrow over 5%. Our patient is an additional evidence to the hypothesis of the common lymphohematopoietic progenitor.

Anemia, Refractory↗

PubMedDate not supplied

[Clinico-pathological characteristics, response to therapy and survival of patients with bone marrow infiltration by Hodgkin's lymphoma].

In a group of 246 patients with Hodgkin's lymphoma we found bone-marrow involvement in 25 patients (10.2%). Clinico-pathological characteristics, response to therapy and survival of those patients were compared with control group of 25 patients in clinical stage ill. Most of the patients with bone marrow involvement were older males, with "B" symptoms, bulky disease, peripheral blood cytopenias and elevated serum alkaline phosphatase. Bone marrow was diffusely infiltrated by Hodgkin's lymphoma in 17 and focally in 8 patients. Marrow fibrosis was noted in 19 patients. Histological type of the disease in affected lymph nodes was not a predictive factor for marrow involvement. Intensive combined chemotherapy was given to 21 patients, and 38% of them achieved prolonged complete remission. The four-year overall survival of 21 treated patients was significantly shorter than in the control group (31.3% vs. 75%, p=0.002). Our study showed that marrow involvement by Hodgkin's lymphoma indicated unfavourable prognosis, particularly in patients with marked splenomegaly and hepatomegaly and elevated serum alkaline phosphatase.

Adult↗

PubMedDate not supplied

[Sezary's syndrome (case report)].

The Sezary's syndrome is a lymphoproliferative disorder from the group of chronical lymphocytic leukaemia originated from the T-cell lineage. Authors are presenting the patient with Sezary's syndrome for the first time ever diagnosed in the Institute of Haematology. The patient, 58 years old got ill in summer 1989. with the symptoms of strong itch and erythematous papullas over the sin. In April 1990. he came to the Institute of Hematology where he was diagnosed as a Sezary's syndrome case on the evidence of generalized erythrodermia, identification of lymphocytes with cerebriform nucleus in peripheral blood and membrane-marker analysis that showed aberant post-thymic proliferation of T-lymphocytes in bone marrow. The patient wastreated with a polychemiotherapy and with "electron beam" therapy with temporary improvement, but died in January 1991 with sepsis and a hepathorenal syndrome.

Humans↗