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Biomedical subjects

P Groff

Publications and source records attributed to P Groff.

34 records · Page 2Linked to original sources
PubMed1979

Pathological cell aggregates in bone marrow cultures from patients with various hematological diseases.

Non-clonal growth of macroscopic cell aggregates in methylcellulose cultures of abnormal marrow is described. They were seen in all patients with Graft-versus-Host Disease, graft rejection, and autoimmune disease presumably directed against hemopoietic cells, we found them in 35% of patients with primary hematological neoplasias and rarely in patients with solid tumors. They were never encountered in 80 healthy controls. The aggregates originated from small cell clumps which sedimented with the "buffy coat" in contrast to normal bone marrow particles. They contained tumor cells, grafted myeloid cells, or target cells of autoimmune disease in association with a widely varying amount of macrophages. Preliminary results suggest that the frequency of macrophages within the aggregates correlates inversely with the aggressiveness of the clinical condition. We propose that appearance of such aggregates in an indicator of immune activation; we expect that further quantitation of the phenomenon will reveal important clinical correlations and provide a model for the study of host defense to "foreign" cells.

Anemia, Hemolytic, Autoimmune↗
PubMed1977

[Allogenic bone marrow transplantation in severe aplastic anemia and refractory acute leukemia. 35 cases].

23 patients with severe aplastic anemia have been treated. 10 marrow grafts between HLA matched siblings were performed after conditioning with cyclophosphamide. 5 are still alive at over 7 months to over 2 1/2 years after grafting. 3 are hemopoietic chimeras and 2 show autologous marrow reconstitution. 13 patients were treated by ALG with or without transfusion of allogeneic marrow. 9 are still alive with partial to complete autologous hemopoietic reconstitution at over 1 month to over 4 years. It is concluded that in many instances aplastic anemia is an autoimmune disease rather than a primary failure of the hemopoietic stem cells. In acute leukemia allogeneic marrow grafts still pose enormous problems and only one of our 12 grafts has become a longterm survivor.

Acute Disease↗
PubMed1976

Failure of isogeneic marrow engraftment in a patient with acute leukemia.

Marrow transplantation was carried out in a patient with acute myelogenous leukemia using a healthy identical twin as donor. The patient was prepared for grafting with combination chemotherapy, high dose cyclophosphamide, and 1,000 rad total body irradiation. Repopulation of marrow did not occur despite a second marrow infusion 21 days following the first attempt. The patient died 44 days after the initial marrow infusion. To our knowledge this is the first patient transplanted from an identical twin who did not achieve engraftment after high dose chemotherapy and supralethal irradiation. The survival time was well in excess of that required for evaluation of engraftment. The possible mechanisms for graft failure are discussed. The use of prophylactic interferon against viral infections is considered as the most likely cause of failure of engraftment.

Bone Marrow Cells↗
PubMed1976

[Graft versus host disease, a little known complication of blood transfusion].

A case of fatal graft-versus-host disease (GvHD) following blood transfusions is reported. This relatively rare complication of blood and bloodcomponent replacement therapy may occur in recipients with decreased cellular immunity, which may be due to the underlying disease, chemotherapy or radiation therapy, or a combination of both. GvHD is a result of active proliferation of transfused immunologically competent cells which attack host organs. This complication can be prevented by irradiating transfusions with 1500 rads in vitro.

Adult↗
PubMed1976

Autologous marrow recovery following allogeneic marrow transplantation in a patient with severe aplastic anemia.

A 45-year-old woman with severe idiopathic marrow failure was prepared for marrow transplantation by administration of cyclophosphamide (cy) 50 mg/kg on each of four successive days. She then received an intravenous infusion of 20 X 10(9) nucleated marrow cells from an HL-A matched and mixed lymphocyte culture (MLC) non-reactive sister. There was evidence for minimal marrow recovery in 1-2 months and a second marrow infusion was carried out 69 days after the first without additional immunosuppression. There was a continued slow recovery of peripheral blood counts with complete reconstitution of erythropoiesis, return of the white blood cell count to between 3 and 4000/mm3, with 50% granulocytes, and platelets to 60--70,000/mm3, 11 months after the initial grafting attempt. Red cell antigens and gamma globulin allotypes were of recipient type. The MLC and the indirect cell mediated lympholysis (CML) test became positive possibly indicating cellular sensitization to non HL-A antigens. This report of a patient with severe marrow failure documents autologous recovery of marrow function after receiving a large dose of cy and allogeneic marrow. The implications of this are discussed.

Anemia, Aplastic↗
PubMed1976

[Thrombocyte replacement with HL-A typed non-related single donors].

Thrombocytopenic patients who have become refractory to platelet transfusions from random donors will usually respond to HL-A compatible platelets with normal increments and hemostatic improvement. In our experience with unrelated HL-A typed donors, HL-A identity is unnecessary. Donors without excess antigen are compatible and even many donors with 1 excess antigen can be used, at any rate in immunosuppressed patients. Thus successful substitution of platelet becomes possible in refractory patients when family donors cannot be used.

Anemia, Aplastic↗
PubMed1975

[Experiences with allogeneic and isogeneic bone marrow transplantations in aplastic anemia and acute leukemia].

A report is presented on 13 clinical bone marrow transplantations (BMT). Six of 6 patients with refractory aplastic anemia are presently alive with partial to complete hemopoetic reconstitution now lasting up to 3 years and more. It is shown that after immunosuppression with ALG and cyclophosphamide, followed by BMT, the patient's own hemopoetic system can recover. Two failures occurred in patients over 50 years of age and in one who had donor transfusions before the graft. Two of 4 patients grafted for acute refractory leukemia are alive, one being in complete remission on day 140. One died from interstitial pneumonia and one had no take although the donor was an identical twin. This patient had been on a maintenance program which included active immunotherapy.

Adolescent↗
PubMedDate not supplied

Short insertion in a hemoglobin chain: Hb Esch, an unstable alpha1 variant with duplication of the sequence Ala65-Leu-Thr-Asn68.

Hemoglobin (Hb) Esch, is an alpha1 variant, expressed at less than 5%, resulting from the duplication of the 12 nucleotides corresponding to CD65 through 68. The effect of this insertion is the repetition of the sequence Ala-Leu-Thr-Asn, which corresponds to the last turn of helix E. In this variant the presence of a one-turn elongated helix E causes instability and increased ligand affinity. Hb Esch was characterized by DNA sequencing and confirmed by electrospray mass spectrometry. Functional studies were performed by flash photolysis measurements on a fraction isolated by flatbed isoelectric focusing, which was enriched in the abnormal hemoglobin. Similar to other alpha chain variants due to short insertion (or deletion), Hb Esch probably results from a slipped mispairing mechanism. The stability of such modified proteins depends upon the region which is added or deleted and usually is more stable when involving a flexible loop or complete helix turn(s) near by.

Adult↗
PubMedDate not supplied

HbSC hemoglobinopathy suspected by chest x-ray and red blood cell morphology.

Thorax scan was performed for elucidation of a pulmonary problem in a Nigerian immigrant. The aspect of the vertebrae suggested sickle cell disease, of course without specification of the genotype. Routine hematological tests seemed compatible with an HbSC disease, showing typical laboratory features, namely a significant proportion of hyperchromic RBC, corresponding to secondary, non hereditary spherocytosis, presence of numerous target cells and occasional HbC crystals on Pappenheim stained blood films. The diagnosis of HbSC disease was confirmed by HPLC, iso-electric focusing and citrate agar electrophoresis of hemoglobin and by reverse phase HPLC of globin-chains. This case illustrates the importance of screening for hemoglobin anomalies as it is performed in a multiethnic country such as the Grand Duchy of Luxembourg

Adult↗