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Results for “myeloproliferative disorders”

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At least 19 recordsLinked to original sources
PubMed1977

Association of breast cancer with myeloproliferative disorders.

Three cases of myeloproliferative disorders in patients with breast cancer are described. The first patient developed acute myeloblastic leukemia 26 years after her initial breast cancer; the second patient developed chronic myelogenous leukemia three years after the diagnosis of breast cancer; the third patient had polycythemia vera for nine years before cancer of the breast was noted. The literature dealing with the association of cancer and myeloproliferative disorders is reviewed. Possible explanations for this association are considered.

Acute Disease↗
PubMed1978

Immunologic dysfunction in the myeloproliferative disorders.

Forty patients with various types of myeloproliferative disorders were evaluated immunologically. Serum immunoglobulin levels were within the normal range in most patients and no monoclonal gammopathies were detected. Serum C'3 levels were decreased in 19 of 40 (48%) patients. The response of peripheral blood lymphocytes to phytohemagglutinin was decreased in 26 of 40 (65%) and to pokeweed mitogen in 18 of 28 (64%) patients studied. Lymphocytes from patients with polycythemia vera were least affected. Unstimulated lymphocytes from some patients demonstrated markedly increased thymidine uptake activity. Despite the diminished mitogenic response, only 2 of 33 patients (6%) were anergic by intradermal skin testing. There was no association between depressed lymphocyte response and recent chemotherapy except in chronic myelogenous leukemia where 6 of 8 patients were receiving cytotoxic therapy when studied. These observations suggest that most of our patients with myeloproliferative disorders have abnormal cellular responses in vitro, but that delayed hypersensitivity and humoral responses are minimally affected.

Adult↗
PubMed1978

Granulopoiesis in chronic myeloproliferative disorders in children.

Four children with chronic myeloproliferative disorders (three with Philadelphia [Ph1] chromosome-positive chronic myelogenous leukemia [CML] were studied with soft agar culture at diagnosis (before therapy) in an attempt to define abnormalities in granulopoiesis. The three patients with CML had elevated peripheral blood golony-forming cells (CFCs) and/or normal or decreased bone marrow CFCs (in those studied). Colony-stimulating activity (CSA) was markedly decreased or absent at diagnosis in all three. Maturation of myeloid cells eithin the colonies in agar was normal, indicating that no block in myeloid maturation was present. These findings are in general agreement with results previously reported in untreated adults with Ph1 chromosome-positive CML and further define the similarity with the adult form of the disease. One Ph1 chromosome-negative patient with a clinically similar chronic myeloproliferative disorder was studied and had similarly elevated peripheral blood CFCs. She had normal CSA with a similarly high WBC count. This finding was unexpected and suggests that, unlike the patients with CML, her monocytes were capable of elaboration CSA. This difference might prove helpful in the classification of this type of disorder in cases where the Ph1 chromosome abnormality is not present.

Adolescent↗
PubMed1977

Ultrastructure of unusual cytoplasmic inclusions in a case of myeloproliferative disorder.

An unusual case of myeloproliferative disorder in which mature and immature myeloid cells were found to contain unusual cytoplasmic inclusions is reported. By light microscopy, these inclusions could be observed with Wright's stain. By electron microscopy they appeared to consist of a pale, finely granular material without surrounding membranes. Although their exact nature remains unknown, we speculate that they may represent the results or products of autophagocytosis of a portion of degenerated cytoplasm.

Aged↗
PubMed1977

Carcinoma of the lung presenting with a myeloproliferative disorder. A report of two patients.

A myeloproliferative disorder, similar to agonogenic myeloid metaplasia, has been described in patients with cancer. Two patients with carcinoma of the lung are described who presented with such a disorder but in whom there was no evidence of bone marrow fibrosis and in one of whom there was no evidence of bone marrow infiltration by tumor. The possible mechanism of this association is discussed.

Aged↗
PubMed1979

Abnormalities of platelet adenine nucleotides in patients with myeloproliferative disorders.

Platelet aggregation and adenine nucleotides in platelets have been studied in thirteen patients with myeloproliferative disorders. ADP induced aggregation was abnormal in two patients, but collagen induced aggregation was impaired in 11 patients. The concentrations of ATP and ADP in resting platelets in the patients with abnormal aggregation were significantly less than those in normal subjects. Marked reduction of the amounts of both nucleotides released into plasma was also observed after stimulation of collagen in these patients. Platelets in the patients with normal functions contained almost normal amounts of adenine nucleotides. We discussed the relationship between platelet dysfunction and adenine nucleotides in platelets of myeloproliferative disorders and concluded that platelet dysfunction was mainly attributable to reduction of releasable ADP.

Adenine Nucleotides↗
PubMed1977

Occurrence of acute leukaemia in myeloproliferative disorders.

In a series of 306 cases of myeloproliferative disorders followed over a period of 21 years, 18 cases of well-documented acute leukaemia were encountered. Leukaemias were either acute myeloblastic or myelomonocytic and occurred from 6 months to 20 years after the initial diagnosis. Onset was relatively abrupt and the course rapidly fatal with with a median survival of 4 weeks. Due to the prolonged preleukaemic phase, it was possible to carry out a variety of clinical and laboratory observations. While no consistent features were noted, dysplastic haemopoiesis, a fall in leucocyte alkaline phosphatase activity, presence of Pelger-Hüet anomaly and other abnormalities suggest a disturbance in granulocytic maturation. These findings suggest that, following an initial injury to a pleuripotential haemopoietic stem cell, a prolonged 'latent' period occurs and, due to exposure to additional injurious agents or to a lack of cell regulating factors, acute leukaemia develops.

Adult↗
PubMed1977

Changes in distribution of platelet membrane glycoproteins in patients with myeloproliferative disorders.

Glycoproteins have been discovered to be important to platelet function both in normal and pathological states. We have studied membrane glycoprotein patterns in 16 patients with various myeloproliferative disorders. There was an abnormal ratio of glycoprotein I:glycoprotein IV in patients with myeloproliferative disease compared with controls. There was no discernible correlation between glycoprotein pattern and aggregation response or platelet count, but patients with megathrombocytes had higher values for glycoprotein IV than those without megathrombocytes. These experiments suggest that patients with myeloproliferative disorders may have alterations in membrane glycoproteins that could alter platelet function.

Blood Coagulation Tests↗
PubMed1979

Altered arachidonate metabolism by platelets in patients with myeloproliferative disorders.

Platelet lipoxygenase and cyclo-oxygenase pathways were investigated by the incubation of 1(-14) C-arachidonic acid with washed platelets in 33 patients with myeloproliferative disorders, including 14 patients with chronic myeloid leukemia (CML), 12 with polycythemia vera (PV), 4 with essential thrombocythemia (ET), and 3 with myelofibrosis (MF). In patients with MF and CML, mean activities of the lipoxygenase pathway were significantly lower when compared with normal controls (p less than 0.001 and p less than 0.01, respectively). When a normal range of the activity was defined as mean +/- 2 SD, all patients with MF, 8 with CML, 6 with PV, and 1 with ET showed decreased lipoxygenase activities, while activities of the cyclo-oxygenase pathway were decreased in one of each patient with CML, PV, and ET. In 4 of 10 patients with a selective lipoxygenase deficiency, platelets were aggregated by lower concentrations of arachidonic acid than those necessary to induce normal platelet aggregation. It is suggested that the lipoxygenase activity could modulate platelet functions through its effect on arachidonate metabolism by the cyclo-oxygenase pathway and that a selective lipoxygenase deficiency could offer a mechanism for hyperfunction of the platelet, which may lead to a thrombotic tendency, one of the common features of myeloproliferative disorders.

Adult↗
PubMed1979

Characterization of the platelet prostaglandin D2 receptor. Loss of prostaglandin D2 receptors in platelets of patients with myeloproliferative disorders.

Prostaglandin (PG) D(2) is synthesized in platelets at concentrations which could inhibit aggregation via activation of adenylate cyclase. To more directly define platelet-PG interactions, a binding assay has been developed for platelet PG receptors with [(3)H]PGD(2) as ligand. [(3)H]PGD(2) binding to intact platelets was saturable and rapid with the ligand bound by 3 min at 20 degrees C. PG competed with the [(3)H]PGD(2) binding site with a potency series: PGD(2) (IC(50) = 0.08 muM) >> PGI(2) (IC(50) = 2 muM) > PGE(1) (IC(50) = 6 muM) > PGF(2alpha) (IC(50) = 8 muM). Scatchard analysis of binding data from six normal subjects showed a single class of binding sites with a dissociation constant (K(d)) of 53 nM and 210 binding sites per platelet. This PGD(2) receptor assay was then used to study platelets from five patients with myeloproliferative disorders (polycythemia vera, essential thrombocythemia, and chronic myelogenous leukemia), as over 90% of these patients have platelets resistant to the effects of PGD(2) on aggregation and adenylate cyclase activity (1978. Blood.52: 618-626.). In the presence of 50 nM [(3)H]PGD(2), the patients' platelets bound 7.1+/-2.9 fmol ligand/10(8) platelets compared with 15.1+/-1 fmol/10(8) platelets in normals, a decrease of 53% (P < 0.01). Scatchard analysis showed that the K(d) of [(3)H]PGD(2) binding (33 nM) was comparable to normal platelets, which indicates that the decreased PGD(2) binding in these platelets represented fewer receptors rather than altered affinity of the ligand for the binding site. The 53% decrease in [(3)H]PGD(2) binding correlated with a 48% decrease in PGD(2)-activated platelet adenylate cyclase. The characterization of the platelet PGD(2) binding site provides further direct evidence that there are at least two PG receptors on platelets, one for PGE(1) and PGI(2), and a separate receptor for PGD(2). Direct binding analysis will be a useful tool for studying the role of PG in regulating platelet function, as demonstrated by the selective loss of PGD(2) binding sites in patients with myeloproliferative disorders.

Adenylyl Cyclases↗
PubMed1979

Myeloproliferative disorders terminating in acute micromegakaryoblastic leukaemia.

Two cases of myeloproliferative disorder--one of myelofibrosis with agnogenic myeloid metaplasia and one of chronic granulocytic leukaemia terminating in acute micromegakaryoblastic leukaemia--are presented. The clinical course is described, and results are reported of morphological, cytometric, cytochemical and cytogenetic studies, as well as cell culture of blood cells in soft agar and in fluid medium.

Adolescent↗
PubMed1978

Acute myelofibrosis with peripheral myeloblastosis: an acute myeloproliferative disorder.

Acute myelofibrosis is an uncommon fulminant disorder characterized by pancytopenia, premature myeloid elements in the peripheral blood, and bone marrow fibrosis. We report the case of a 59-year-old man who had acute myelofibrosis and peripheral myeloblastosis clinically suggesting the diagnosis of acute granulocytic leukemia. The disease was unresponsive to cytotoxic drugs or androgens and the patient died five months later. The association of bone marrow fibrosis with large numbers of myeloblasts in the peripheral blood has rarely been reported and suggests a spectrum of morphological changes in acute myeloproliferative disorders, analogous to the merging of chronic myeloproliferative disorders into one another and into leukemic blast crisis.

Bone Marrow↗
PubMed1979

Isochromosome 17 in a patient with a myeloproliferative disorders terminating in eosinophilic leukemia.

A patient is described, who for more than two years had a myeloproliferative disorder which terminated in eosinophilic leukemia. Chromosome analysis revealed an isochromosome 17 in all metaphases of bone marrow cells. This abnormality has now been found in two out of six patients with eosinophilic leukemia investigated by banding techniques, and may therefore have etiologic importance. Chromosome analysis in the hypereosinophilic syndrome has practical value for differentiating malignant and non-malignant disease.

Aged↗
PubMed1977

Myeloproliferative disorders: a paradox of in-vivo and in-vitro platelet function.

A patient with features of a myeloproliferative disorder developed an acute multisystems illness and died. In-vitro platelet aggregation was imparied, but necropsy revealed widespread platelet-rich thromboemboli and multiple organ infarctions. It is suggested that platelets are damaged during disseminated intravascular platelet aggregation (DIPA) and that disaggregation of platelet thrombi and recirculation of platelets give rise to their subsequent hypofunction when tested in vitro.

Aged↗
PubMed1978

Chronic myeloproliferative disorders: improved platelet aggregation following venesection.

Venesection of 10% of whole blood volume or plateletpheresis was performed in nine patients with chronic myeloproliferative disorders and in five normal control subjects. Before venesection, the patients showed impaired platelet aggregation in 33% of tests, most often in response to stimulation with 9 mumol adrenaline. After venesection, the platelet and megathrombocyte counts increased rapidly and excessively in most patients and platelet aggregation improved markedly. In some cases, spontaneous in vitro aggregation was seen at high platelet concentrations. In two patients impaired platelet aggregation with adrenaline was not corrected. The splenic platelet pool is thought to be the probable source of the new platelets.

Adenosine Diphosphate↗
PubMed1979

The colony forming cell in the myeloproliferative disorders and aplastic anaemia.

Bone marrow colony forming cell (CFC) concentration and the proportion of CFC in DNA synthesis were studied in myeloproliferative disorders and aplastic anaemia. Growth patterns of bone marrow cells in agar cultures were able to supplement traditional morphological and clinical criteria in the diagnosis of these haematological conditions. Bone marrow CFC concentration tended to be increased in chronic myeloid leukaemia (CML) and polycythaemia vera (PV), but decreased in myelofibrosis, erythroleukaemia, paroxysmal nocturnal haemoglobinuria (PNH) and the aplastic phase of aplastic anaemia. The proportion of CFC in DNA synthesis was decreased in CML, myelofibrosis and aplastic anaemia, but increased in blastic transformation, PV, PNH and during regeneration from aplastic anaemia. The proportion of CFC in DNA synthesis in bone marrow from patients with CML in blastic transformation was directly related to the percentage of myeloblasts in the bone marrow. CFC kinetics in blastic transformation have been demonstrated to be different from those in acute leukaemia.

Anemia, Aplastic↗