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T Ruutu

Publications and source records attributed to T Ruutu.

At least 55 records · Page 3Linked to original sources
PubMed1988

A new congenital dyserythropoietic anaemia.

A new dominantly inherited dyserythropoietic anaemia is described. In the bone marrow, many of the nonspecific morphological characteristics described in congenital dyserythropoietic anaemias were seen; however, dysmorphic cells were rare. The acidified serum test was positive with one out of 17 sera tested; the negative sera included two that had haemolysed HEMPAS erythrocytes in the acid Ham test. Anti-i-agglutination was negative. No aberrations of red cell membrane protein glycosylation were observed. Serum cholesterol was low. Bilirubin conjugation was deficient but icterus was resolved by treatment with phenobarbital.

Adult↗
PubMed1988

Erythroid colony formation and effect of hemin in vitro in hereditary sideroblastic anemias.

Colony formation by erythroid burst-forming units (BFU-E) and erythroid colony-forming units (CFU-E) and the effect of hemin on colony growth was studied in vitro in three Finnish families with hereditary sideroblastic anemia (HSA). Defective activity of heme synthase has been demonstrated in family A and that of delta-aminolevulinic acid synthase in family B. No biochemical defect has been recognized so far in family C. CFU-E colony growth was defective in seven of the eight persons studied. The formation of BFU-E colonies was normal in family A and increased in family C, whereas of the two members of family B one showed normal and one decreased BFU-E colony growth. Hemin in 30-120 microM concentration increased significantly both BFU-E (p less than 0.01) and CFU-E (p less than 0.005) colony formation in family C. No effect was seen in family A, and in family B the only effect was normalization of the decreased BFU-E colony growth by the highest hemin concentration in one person. This study indicates that differences exist between families with HSA in erythroid colony formation and in response to hemin in vitro, but the low number of investigated members in each family does not permit a conclusive evaluation of the impact of the carrier versus patient status or of sex on the results.

Adult↗
PubMed1988

Monocytic involvement by monosomy 7 preceded acute myelomonocytic leukemia in a patient with myelodysplastic syndrome.

Novel techniques were used to detect which cell lineages were affected by monosomy 7 in a patient who had myelodysplastic syndrome and later developed acute leukemia. The patient had had paroxysmal nocturnal hemoglobinuria for 20 years before developing refractory anemia with excess of blasts. Cytogenetic analysis at the myelodysplastic stage disclosed monosomy 7 in bone marrow mitoses. Restriction fragment length polymorphism analysis of fractionated white blood cells with the chromosome 7-specific probes MetH and MetD revealed that blood monocytes and most bone marrow erythroblasts but not blood granulocytes or lymphocytes were affected by monosomy 7. The patient later developed acute myelomonocytic leukemia with blast cells positive for markers of the myelomonocytic lineage but negative for granulocytic markers in a standard surface marker analysis. The leukemic blast cells had monosomy 7 as determined by direct cytogenetic investigation. Thus, the monocytes were found to be affected by monosomy 7 in this patient 8 months before her myelodysplastic syndrome progressed to acute myelomonocytic leukemia, and the affected cells had the same biologic markers at both stages of the disease.

Anemia, Refractory, with Excess of Blasts↗
PubMed1987

Monosomy 7 in granulocytes and monocytes in myelodysplastic syndrome.

Monosomy for all or part of chromosome 7 in bone marrow mitoses of some patients with myelodysplastic syndrome or acute nonlymphocytic leukemia has been associated with a defect in granulocyte function. To study which blood-cell lineages are affected by the monosomy, we used chromosome 7-specific DNA probes in Southern blotting experiments on DNA derived from specific cell fractions isolated from the blood of five patients. As judged by the presence or absence of two different alleles for restriction-fragment-length polymorphisms, lymphocytes of all five patients were shown to have two different chromosomes 7. Granulocytes were affected by the chromosomal abnormality in four patients (No. 1, 2, 4, and 5) and unaffected in one (No. 3). Chemotaxis was normal in Patient 3 and impaired in Patients 4 and 5. Monocytes were affected by the monosomy in two of three patients (No. 2 and 3) and mainly unaffected in one (No. 1). Thus, the granulocytes and monocytes were affected differently in different patients. We conclude that mature blood cells are derived from abnormal progenitors and that there may be heterogeneity in the involvement of different cell lineages in different patients with myelodysplastic syndrome or acute nonlymphocytic leukemia. There is an association between DNA loss and functional impairment.

Acute Disease↗
PubMed1987

Constitutional translocation t(3;6)(p14;p11) in a family with hematologic malignancies.

We describe a family with an inherited constitutional balanced translocation t(3;6)(p14;p11) and hematologic malignancies. Of two proven translocation carriers, one had acute myeloid leukemia and the other had myelofibrosis. A third member who had died of acute leukemia was a possible translocation carrier (chromosome analysis had not been performed). Five healthy translocation carriers were detected. Neither the translocation nor additional hematologic malignancies were found outside the nuclear family. It could not be definitely clarified if this constitutional translocation predisposes to hematologic malignancies. Breakpoint 3p14 has previously been implicated in recurrent cancer-associated rearrangements but 6p11 has not. We suggest that other investigators look for involvement of these breakpoints in cancer patients.

Acute Disease↗
PubMed1987

Colony formation by megakaryocytic progenitors in essential thrombocythaemia.

Colony formation by megakaryocytic progenitors (CFU-M) from the blood or bone marrow of 12 patients with essential thrombocythaemia was studied in vitro with the methyl cellulose assay. When the cultures were stimulated with plasma from a patient with aplastic anaemia and with phytohaemagglutinin stimulated conditioned medium (PHA-LCM), the patients showed a significant trend towards higher circulating CFU-M numbers when compared with the controls; three of the patients exceeded our normal range. In the bone marrow cultures there were no differences in the number or morphology of megakaryocytic colonies between the patients and the controls. When normal human plasma was the only source of colony stimulating activity, 11 out of 12 patients, but none of the controls, showed megakaryocytic colony formation. The same patients also had 'spontaneous' erythroid colony growth without the addition of exogenous erythropoietin into the cultures. Only one of the patients with essential thrombocythaemia (ET) had normal megakaryocytic and erythroid colony formation. The present study shows that, in most patients with ET 'spontaneous' CFU-M colony formation occurs in suboptimal culture conditions, a phenomenon obviously analogous to the spontaneous erythroid colony formation seen in the myeloproliferative disorders.

Adult↗
PubMed1987

Haem arginate as a treatment for myelodysplastic syndromes.

We report on two patients with a myelodysplastic syndrome in whom the blood cell counts markedly improved during treatment with haem arginate. One patient received haem arginate only, the other haem arginate in combination with low dose androgen. The drug was given as weekly infusions of 2-3 mg/kg body weight for 8-12 weeks. In one patient the percentage of ringed and other abnormal sideroblasts in the bone marrow was clearly reduced as a result of the treatment. In both patients the effect on blood cell counts lasted for several months after the cessation of the haem arginate treatment. Eleven other patients showed no clear response. No adverse effects of the infusions were observed. Further studies on the possible therapeutic role of haem arginate are indicated.

Adult↗
PubMed1987

Graft-versus-leukaemia activity associated with cytomegalovirus seropositive bone marrow donors but separated from graft-versus-host disease in allograft recipients with AML.

To elucidate whether a relationship existed between bone marrow donor cytomegalovirus (CMV) immune status and the probability of staying in remission after transplantation, a retrospective multicentre analysis was performed in 69 patients who received allogeneic bone marrow transplantation during relapse or second remission of AML, or second remission of ALL. None of 12 AML patients with CMV seropositive donors had posttransplant relapse, in contrast to 7 of 10 AML patients with seronegative donors. Kaplan-Meier estimates of the 2-yr probability of staying in remission for the two groups were 100% and 0%, respectively (p less than 0.0005). This effect was independent of disease stage, donor and recipient age, recipient pretransplant CMV immune status and the occurrence of posttransplant CMV infection in recipients, and was not mediated through an increased occurrence of overt graft-versus-host disease (GvHD) in recipients with CMV seropositive donors. The increased probability of staying in remission was associated with an increased probability of 3-yr disease-free survival (p less than 0.01). No similar effect was observed in patients with ALL. This study may suggest an allograft-versus-leukaemia effect in AML, associated with CMV seropositive donors, which seems separate from GvHD and independent of the occurrence of posttransplant CMV infection.

Adolescent↗
PubMed1987

Correlation between bone marrow karyotype and the occurrence of erythroblast micronuclei and nuclear budding in patients with myelodysplastic syndromes.

147 patients with myelodysplastic syndromes were investigated for the presence of micronuclei and nuclear budding in bone marrow erythroblasts. The patients were divided into subgroups on the basis of bone marrow karyotype, 31 healthy bone marrow donors constituted a control group. Patients with monosomy 7 or 7q- and patients with major karyotypic abnormalities (MAKA) had significantly more erythroblasts with micronuclei and nuclear budding than the control group. Patients with a 5q- chromosome as the sole karyotypic aberration had more micronuclei than the controls. For other patients with MDS the differences were statistically nonsignificant.

Adolescent↗
PubMed1987

An outbreak of invasive aspergillosis in a haematologic unit.

Within a period of 15 months 8 cases of invasive pulmonary aspergillosis were seen in a haematologic unit; 7 of them between January and October 1984. No previous cases of invasive aspergillosis had been encountered during the existence of the unit since 1979. Environmental studies did not prove a single likely source for the fungal spores. Previous window renovation with concomitant fiber deposits on ventilation grids, poor sealing of air filter fittings in patient rooms, occasional ventilation through windows in the ward, and low speed of booster fans in the ventilation system may have created a condition favourable for the entry of Aspergillus fumigatus spores into the patient rooms. Environmental sanitation including cleaning of the ventilation ducts and change of filters in the ventilation system stopped the outbreak. Two sporadic cases have appeared during a follow-up period of 26 months.

Acute Disease↗
PubMed1987

Primary infection with HIV in a severely immunosuppressed patient with acute leukemia.

A 47-year-old female with acute myeloid leukemia received HIV positive platelets during induction chemotherapy. 18 days later, coincident with the recovery of the bone marrow function, she developed an erythematous rash, mild lymphadenopathy, and nausea which disappeared within 10 days. A week later mild CSF pleocytosis consisting of mature lymphocytes and macrophages together with elevated CSF protein levels (1,080 mg/l) were observed suggesting mild aseptic meningitis, and the HIV was concomitantly isolated from CSF. The CSF abnormalities have improved and the patient is well and in remission after 3 cycles of chemotherapy. This case expands the clinical spectrum of HIV infection to include a primary syndrome during immunosuppression from an unrelated cause.

Acquired Immunodeficiency Syndrome↗
PubMed1987

Invasive pulmonary aspergillosis: a diagnostic and therapeutic problem. Clinical experience with eight haematologic patients.

Eight patients with haematologic malignancies contracted fatal invasive aspergillosis during an outbreak. Five patients were neutropenic. Bronchofiberoscopic examination with microbiology specimen brush and bronchoalveolar lavage yielded Aspergillus fumigatus in only 2/5 patients examined. The specific diagnosis reached during lifetime in 5 patients was based on a combination of invasive procedures (lung biopsy in 2, percutaneous lung puncture in 1), the presence of a lung abscess (3 patients), seroconversion (1 patient), and purulent maxillary sinusitis caused by A. fumigatus together with repeated abundant growth of A. fumigatus in the sputum (1 patient). Six patients received amphotericin B. The infection was temporarily controlled only in 2 bone marrow transplant recipients whose granulocyte counts recovered. In 3/8 patients the pneumonia was of polymicrobial aetiology, Mycobacterium tuberculosis (2 patients), Pneumocystis carinii (1 patient), and Legionella pneumophila (1 patient) being the other microbes involved. 3/4 bone marrow transplant recipients with aspergillosis had been transplanted for chronic myeloid leukaemia, supporting the previously reported association of bone marrow transplantation for chronic myeloid leukaemia and the risk of invasive aspergillosis. Improved diagnostic methods for earlier definitive diagnosis of invasive aspergillosis as well as more efficacious and less toxic antifungal agents are needed to allow early treatment.

Adult↗
PubMed1987

Failure to prevent cytomegalovirus infection by cytomegalovirus hyperimmune plasma: a randomized trial by the Nordic Bone Marrow Transplantation Group.

Bone marrow transplantation recipients who were cytomegalovirus (CMV) seropositive and/or had a CMV seropositive donor were randomized for treatment with CMV hyperimmune plasma (n = 27) or no treatment at all (n = 27). The CMV hyperimmune plasma had neutralization titers greater than 250 and enzyme-linked immunosorbent assay titers greater than 18,000. Plasma (200 mg/kg body weight) was given on four occasions (during 2 days) from day 3 to day 76 after transplantation. Patient characteristics were similar in the two groups. After transplantation, the median CMV titers increased with greater than 100% in the group receiving the CMV plasma and decreased to less than 50% in the controls (p less than 0.01). Asymptomatic CMV infections occurred in 26% of the patients in the plasma group and 33% of the controls. The frequency of patients with symptomatic CMV infections was also the same in the two groups (51% vs 33%). Three patients each in the two groups developed CMV-associated interstitial pneumonitis. Patient survival and causes of death were similar in the two groups. To conclude, no beneficial effect of CMV hyperimmune plasma was seen in patients at high risk of developing CMV infections.

Antibodies, Viral↗
PubMed1987

Molecular characterization of chromosome 7 long arm deletions in myeloid disorders.

Partial deletion of the long arm of chromosome 7 is a common abnormality in the bone marrow cells of patients with myelodysplastic syndrome (MDS) or acute nonlymphocytic leukemia (ANLL). This study was undertaken to characterize the chromosome breakpoints in molecular terms and to determine if hemizygosity or submicroscopic deletions occur in patients without any cytogenetically detectable abnormality of chromosome 7. We studied restriction fragment length polymorphisms with 10 chromosome 7-specific DNA probes in separated WBC fractions. No molecular abnormalities occurred in lymphocyte-derived DNA. Several probes located in band 7q22 or distally thereof detected deletion of one allele in granulocyte-derived DNA from all four patients with chromosome 7 long arm deletion. In the granulocytes of one patient heterozygosity for the T cell receptor beta chain gene (in band 7q35) indicated that the deletion was interstitial. NJ-3, a proalpha2(I)collagen gene probe (in band 7q21-22) detected heterozygosity in the granulocytes of one patient. No hemizygosity or deletions were found in four patients with two normal chromosomes 7. These results confirm that mature granulocytes but not lymphocytes are derived from the abnormal clone. Interstitial deletions exist, and the extent of deleted genomic material varies among patients.

Acute Disease↗