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Biomedical subjects

L Chrobák

Publications and source records attributed to L Chrobák.

At least 37 records · Page 2Linked to original sources
PubMed1998

Red cell distribution width (RDW) as a marker of disease activity in patients with hairy cell leukemia.

UNLABELLED: Red cell distribution width (RDW) was examined in 18 patients with hairy cell leukemia (HCL) treated with 2-chlorodeoxyadenosine (2-CdA), in 5 patients treated with Interferon alpha (IFN-alpha) and in 9 patients subjected to splenectomy. Out of 18 patients treated with 2-CdA one patient was excluded of the study because of association of HCL with acquired sideroblastic anemia. In the remaining 17 patients the mean value of RDW before therapy was 18.8% (range 13.5% to 25.0%) and dropped after successful therapy after 6 to 12 months to the mean value of 13.6% (range 11.2% to 17.9%) and after 18 months to 13.4% (range 12.6% to 14.7%) (p = 0.00015 and p = 0.00049 respectively). The hemoglobin level increased from the mean value of 119 g/l (range 99 g/l to 157 g/l) before therapy to the mean value of 145.9 g/l (range 127 g/l to 172 g/l) after 6 to 12 months and after 18 months to 147.8 g/l (range 132 g/l to 168 g/l) (p = 0.000017 and p = 0.00036 respectively). The same trend was observed in the group of patients treated with IFN-alfa. The RDW decreased from the mean value of 21.3% (range 18.8% to 28.7%) to the mean value of 15.3% (range 12.4% to 16.7%), (p = 0.031). The hemoglobin level increased in this group of patients from the mean value of 115 g/l (range 98 g/l to 127 g/l) to the mean value of 136 g/l (range 127 g/l to 146 g/l) (p = 0.031). In 9 patients in complete hematologic remission 34 to 293 months after splenectomy the mean value of RDW was 13.9% (range 13.0% to 15.5%). CONCLUSION: Increased RDW in HCL is associated with active disease and is reversible after successful therapy. This phenomenon has not been reported in the literature yet. Preliminary results show that the increase of RDW may be due to the dyserythropoiesis.

2-Chloroadenosine↗
PubMed1998

[Indications for splenectomy].

Splenectomy may be indicated for either of two medical conditions: 1. for diagnostic reasons in order to clarify the undiagnosed process localized in the spleen and for the staging of malignant lymphomas, 2. or for therapeutic purposes in order to remove the pathologically exaggerated physiological function of the spleen residing in pooling, sequestration and destruction of blood corpuscles and to remove benign and malignant processes localized in the spleen. The spleen also may have to be removed because of traumatic or spontaneous ruptures causing intra-abdominal hemorrhage.

Humans↗
PubMed1998

Dyserythropoietic changes and sideroblastic anemia in patients with hairy cell leukemia before and after therapy with 2-chlorodeoxyadenosine.

Active hairy cell leukemia is associated with an increase of RDW (Red Cell Distribution Width) which normalizes after successful therapy with 2-chlorodeoxyadenosine (2-CdA). To clarify this phenomenon, bone marrow films performed before therapy with 2-CdA and after its successful completion were subjected to careful evaluation. Dyserythropoietic changes were present in 5 out of 17 patients before the therapy with 2-CdA. In 2 patients the changes were only slight, characterized by irregularities of the shape of nucleus and nuclear contour, in the remaining 3 patients the changes were marked, represented by nuclear lobulation, karyorrhexis and binuclearity, with the presence of ringed sideroblasts in one of them. After therapy with 2-CdA complete hematologic remission was achieved in 4 patients with disappearance of dyserythropoietic changes and normalization of RDW values. In the last patient with ringed sideroblasts despite complete remission with disappearance of tumoral cells in the bone marrow as demonstrated by immunohistochemical analysis of trephine bone marrow biopsy with monoclonal antibody DBA 44 the condition deteriorated, RDW remained unchanged, the sideroblastic anemia progressed.

Adult↗
PubMed1997

Long-term results in hairy cell leukemia treated with 2-chlorodeoxyadenosine.

We treated 19 patients with hairy cell leukemia (HCL) with 2-chlorodeoxyadenosine. 15 patients followed up at least 6 months were evaluated. The follow up period varied between 6 months and 37 months (median, 19 months). 8 patients were previously treated. The overall response in 15 evaluable HCL patients was 100%, with 87% complete hematological remissions including three patients with retroperitoneal and mediastinal lymphadenopathy and one patient with leukemic infiltrates of the cornea: 13% of patients achieved partial hematological remission. Soluble interleukin-2 receptor (sIL-2R) considered as a reliable non-invasive marker of HCL tumor burden dropped from the median of 1350 pM/ml (range 188 to 9000 pM/ml) to the median of 84.3 pM/ml (range 37 to 382 pM/ml) RdW which reflects the anisocytosis of red cells decreased after therapy from the median of 20.6% (range 13.1-25.0%) to the median of 13.7% (range 12.4-16.3%).

Adult↗
PubMed1997

[A fully implantable port system for venous access. Personal experience with 91 patients].

A fully implantable venous port system with a cannula inserted into the central venous system was implanted to 91 patients. The system was used for the administration of different types of intravenous preparations, incl. transfusions of erythrocyte mass and parenteral nutrition. With in the framework of autologous transplantation via the port system haematopoietic progenitor cells from the peripheral blood stream were administered. Part of the introduced systems was used for monitoring of the central venous overpressure and for collecting blood samples. The system was introduced on an average for 289 days, with a median of 245 days, range 82-872 days. During the implantation seven complications developed, in three instances the port was removed prematurely. During treatment 16 complications were observed, which in six instances led to early explantation of the venous port. The number of complications in total amounted to 0.87 per 1000 treatment days. The system was well tolerated by the patients because of its functional effectiveness and minimal cosmetic changes, and because it is not demanding it was also accepted well by the nursing staff.

Catheterization, Central Venous↗
PubMed1997

[Favorable effect of splenectomy on anemia in 3 siblings with type II congenital dyserythropoietic anemia (HEMPAS). (Ultrastructural changes in erythrocytes after splenectomy)].

Congenital dyserythropoietic anemia type II (CDA-type II) (HEMPAS) was reported in three siblings. CDA-type II was associated with marked hepatosplenomegaly and siderosis of both organs. All three sibling developed cholelithiasis with choledocholithiasis and obstructive jaundice in two of them. Anemia showing hemolytic component with sequestration of erythrocytes in the spleen was corrected after splenectomy without the need of blood transfusions during the follow up period of 20, 11 and 11 years. Ultrastructural investigation of the erythrocytes after splenectomy revealed increased number of erythrocytes showing the double membrane phenomenon.

Adult↗
PubMed1996

[An unusual course in hairy-cell leukemia with marked abdominal lymphadenopathy, leukemic infiltration of the cornea and skin changes].

The authors describe a female patient suffering from hairy-cell leukaemia. Already at the onset of the disease, apart from marked splenomegaly, sonography revealed marked retroperitoneal lymphadenopathy. During the subsequent course skin changes developed such as vasculitis and leukaemic infiltrates of the cornea on both eyes. The patient was successfully treated with 2-chlorodeoxyadenosine (2-CdA, Leustatin).

Cornea↗
PubMed1996

[Heparin-induced thrombocytopenia].

Heparin induced thrombocytopenia (HIT) is the result of platelet activation and it is either nonimmunologically (type I) or immunologically mediated (type II). HIT is the most frequent drug induced immune thrombocytopenia. It is completely different from all other forms of immune thrombocytopenia, because it can be accompanied by serious thromboembolic complication because of its serious clinical consequences must be prompt and very intensive.

Anticoagulants↗
PubMed1996

Serum soluble IL-2 receptor as a reliable and noninvasive marker of disease activity in patients with hairy cell leukemia.

Hairy cell leukemia is a chronic lymphoproliferative disorder of B-cell lineage. Malignant cells express the interleukin-2 receptor (IL-2R) which is released in vitro as well as in vivo. The sera of patients with hairy cell leukemia contain elevated levels of this soluble receptor (sIL-2R). Sera of 24 patients with hairy cell leukemia were tested for sIL-2R. In 9 patients treated with 2-chlorodeoxyadenosine an improved clinical status was associated with decreasing serum sIL-2R. The maximal rate of decrease of sIL-2R level was observed within the second and the third week after the therapy initiation. Patients with disease progression had an increase in serum sIL-2R level. Our results suggest that serial measurement of sIL-2R level can be used as a reliable, noninvasive means to assess the disease activity and its response to therapy.

Adult↗
PubMed1996

Clinical significance of soluble interleukin-2 receptor.

The measurement of soluble interleukin-2 receptor in serum has been shown to be useful in a variety of clinical conditions. In autoimmune disorders sIL-2R level correlates with the disease activity and reflects in-vivo immune system activation. Markedly elevated sIL-2R levels were found in certain hematologic malignancies such as adult T-cell leukemia and hairy cell leukemia. In hairy cell leukemia it reflects the tumor burden and response to therapy and can be used as a reliable non-invasive marker of disease activity. The sIL-2R levels at diagnosis predict reliably prognosis in non-Hodgkin lymphoma and in Hodgkin's disease.

Autoimmune Diseases↗
PubMed1996

Resistance to activated protein C as pathogenic factor of venous thromboembolism.

Venous thromboembolism is a serious health problem and a significant cause of morbidity and mortality. It is associated with certain situations such as surgery, trauma, immobilization, obesity, the use of oral contraceptives, pregnancy and puerperium. In addition, genetic factors have been found to be important in the pathogenesis of venous thrombosis and pulmonary embolism. Until recently, the main genetic defects known to be associated with thromboembolism were antithrombin III, protein C and protein S deficiency accounting together for about 10% of the cases. In 1993 Dahlbäck et al. reported a familial thrombophilia due to a previously unrecognized mechanism characterized by an increased resistance to anticoagulant action of activated protein C (APC). APC-resistance is highly prevalent in general population (2-7%) and in its heterozygous form leads to a life-long hypercoagulable state and 5-10 fold increased risk of thrombosis. It is a major basis for venous thromboembolism and is found in 17 to 64% of patients with inherited thrombophilia.

Animals↗
PubMed1995

Hairy cell leukaemia, its therapy and prognosis (historical review with own experiences).

The best treatment protocol for hairy cell leukaemia (HCL) has not yet been defined. Different chemotherapies failed to demonstrate sustained benefit. Splenectomy certainly has been beneficial for many patients but has no effect on bone marrow infiltration by leukaemic cells. The introduction of Interferon-alpha (IFN-alpha), 2-deoxycoformycin (DCF) and 2-chlorodeoxyadenosine (2-CdA) in the therapy of HCL dramatically improved treatment options during the past decade. IFN-alpha does not have curative potential in HCL. 2-CdA seems to be more promising than DCF. Serum IL-2R level can be used as non invasive means to assess disease response to therapy. The predictive reliability of this test remains to be evaluated. The definition of complete remission remains open being greatly influenced by the sensitivity of the methods applied to detect residual disease. The role of the spleen as the reservoir of malignant cells can not be excluded in respect to the relapses of the disease.

Adolescent↗
PubMed1995

[Leukemic infiltration of the cornea in a patient with hairy-cell leukemia].

The authors describe the infiltrations of the cornea in both eyes of a patient (women) who suffered from leukaemia with "long-haired cells". The treatment was successful with 2-chlordeoxyadenosine (Leustatin). Knowledge of leucaemic infiltration in the cornea is accentuated for successful treatment in connection with basic disease.

Cornea↗
PubMed1995

[Structural variants in hemoglobin occurring in the Czech Republic].

The authors present a review of clinical and laboratory findings of seven in the Czech Republic hitherto diagnosed structural haemoglobin variants. Unstable variants are found most frequently: Hb-Köln, Hb-St. Louis, Hb-Nottingham, Hb-E and Hb-Hradec Králové. The variant Hb-Hradec Králové (Hb-HK) or alpha 2 beta 2 115 (G17) Ala-Asp was newly detected. The great instability of Hb-HK chains makes classical diagnosis of Hb-pathy impossible. It was possible to identify it only at a molecular genetic level. A manifestation of Hb-HK instability is also the thalassaemic feature of the disease and the formation of Heinz bodies from free chains. The only representative of haemoglobins with a high oxygen affinity identified in this country was newly detected. It was given the name Hb-Olomouc or alpha 2 beta 2 86 (F2) Ala-Asp. This haemoglobin variant leads to erythrocytosis in father and son and the same clinical manifestations were recently described also in Japan. The last structural variant of haemoglobin found in this country is Hb-M Milwaukee or alpha 2 beta 2 67 (E11) Val-Glu which in our patients is manifested rather by haemolysis with formation of Heinz bodies than classical cyanosis. The cause of instability of Hb-M in our patients is not known. Hb-S was not diagnosed so far in the Czech Republic.

Adolescent↗
PubMed1994

The value of bone marrow biopsy in the prognosis of hairy cell leukemia (HCL).

Histological findings in the bone marrow and their changes in the course of the disease and therapy were evaluated with respect to the prognosis in a group of 32 patients with hairy cell leukemia (HCL). Two types of bone marrow infiltration by hairy cells (HCs), the diffuse type and the interstitial type, respectively were found. The diffuse type of infiltration and the minimal or absent residual hematopoiesis (RH) at presentation were found with statistical significance to be unfavorable prognostic findings when compared with interstitial infiltration and persisting RH (p < 0.01). Reticulin fibrosis is a characteristic finding in HCL and was found in all but 2 patients.

Adult↗
PubMed1994

Red cell ferritin and iron stores in chronic granulocytic leukemia.

Basic red cell ferritin was investigated in 28 patients with different phases of chronic granulocytic leukemia (CGL). Red cell ferritin was significantly decreased in remission after busulphan treatment and significantly elevated in the blast crisis as compared to healthy controls. Bone marrow stainable iron was decreased or absent in 86% of patients in the initial phase at the time of diagnosis and in 92% of those in remission. Red cell ferritin correlated with serum ferritin, however, serum ferritin level remained above normal range during all phases of the disease. A negative correlation between red cell ferritin and hemoglobin (Hb) (r = -0.605, p < 0.001) suggested that red cell ferritin level reflected the rate of iron utilization for heme synthesis. Decreased red cell iron stores observed in the remission may be explained by regression of dyserythropoiesis and by restoration of normal Hb synthesis after busulphan treatment. A progressive dyserythropoiesis in the blast crisis may lead to an increased red cell ferritin level.

Biopsy↗
PubMed1994

[Actilyse (a recombinant tissue-type plasminogen activator] in the treatment of acute myocardial infarct. Comparison of the effects of Actilyse and streptokinase].

The preparation Actilyse--a tissue plasminogen activator prepared by recombination--is an effective fibrinolytic drug. The authors recorded in a group of 33 patients reperfusion in 91%, evaluated on the basis of indirect criteria. In a group of 18 patients treated with streptokinase reperfusion was achieved in 74%. Based on changes of CK and CK-MB values--an earlier rise of values following Actilyse administration and conversely their more marked increase during subsequent sampling after streptokinase administration--it may be assumed that earlier dissolution of the thrombus in the coronary artery occurs after Actilyse administration, as compared with streptokinase. It may be thus assumed that there is also a smaller necrotic focus after Actilyse treatment, as compared with streptokinase. Early re-occlusion--according to indirect indicators--occurred in 8.6% in the Actilyse treated groups, as compared with 5.9% in the streptokinase treated group. The fibrinogen values decline in the Actilyse group to 40% and in the streptokinase group to 28%. Later enhanced new formation of fibrinogen occurs and the fibrinogen values rise to 160% in the Actilyse treated group and to 250% of the initial value in the streptokinase treated group. The elevated fibrinogen value, as compared with the baseline value, persists for the 12 days of the follow-up. No severe spontaneous haemorrhage was recorded, haemorrhagic manifestations were associated with blood sampling and i.v. administration of drugs only. The necessity to administer blood was due to a complication during puncture of the subclavian vein in one patient.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗