ABSTRACT
The paper presents a case of autoimmune hemolytic anemia (AIHA) with warm IgG antibodies associated with Waldenström's disease in which in the stage of compensated hemolysis after treatment, there appeared a severe hemolytic attack induced by transitory cold agglutinins with high thermal amplitude. The case described is a novelty by the intrication of two autoantibody populations which causes autoimmune hemolysis. The difference from other similar associations is discussed.
Subject(s)
Agglutinins/blood , Anemia, Hemolytic, Autoimmune/diagnosis , Autoantibodies/blood , Immunoglobulin G/blood , Waldenstrom Macroglobulinemia/diagnosis , Adult , Anemia, Hemolytic, Autoimmune/etiology , Anemia, Hemolytic, Autoimmune/therapy , Cold Temperature , Combined Modality Therapy , Fatal Outcome , Hot Temperature , Humans , Waldenstrom Macroglobulinemia/complications , Waldenstrom Macroglobulinemia/therapyABSTRACT
The present paper reports on a case of acute lymphoblastic leukemia with t(9q+; 22q-) and surface markers specific of the B and T lymphoid line (hybrid phenotype). The fundamental (genotypical and phenotypical) and practical aspects (nosologic and therapeutical aspects) of this particular subtype of Ph1-positive acute leukemia and hybrid phenotype are discussed.
Subject(s)
Leukemia, Myelogenous, Chronic, BCR-ABL Positive/diagnosis , Precursor Cell Lymphoblastic Leukemia-Lymphoma/diagnosis , Adult , Antineoplastic Combined Chemotherapy Protocols/administration & dosage , Chromosome Aberrations , Chromosomes, Human, Pair 9 , Female , Genotype , Humans , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/blood , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/drug therapy , Leukemia, Myelogenous, Chronic, BCR-ABL Positive/genetics , Phenotype , Philadelphia Chromosome , Precursor Cell Lymphoblastic Leukemia-Lymphoma/blood , Precursor Cell Lymphoblastic Leukemia-Lymphoma/drug therapy , Precursor Cell Lymphoblastic Leukemia-Lymphoma/geneticsABSTRACT
A patient with non-cutaneous lymphocytic lymphoma with leukaemic spread is reported. The large majority of the peripheral blood mononuclear cells formed rosettes with sheep erythrocytes (E), had receptors for the Fc portion of IgG (Fc gamma R) and displayed an unusual enzymatic profile. Part of these cells had also labile bound surface membrane IgG. The leukaemic cells were morphologically characterized as large granular lymphocytes (LGL) and consisted of two different types of cells: lymphoid cells and monocytoid cells. The histopathological diagnosis was T-cell lymphoma, pleomorphic type, diffuse. Despite their morphological heterogeneity, all the proliferative cells had the same immunological phenotype, showed normal ADCC activity but no NK activity. After three months, without specific treatment, the surface pattern changed: the cells resembled mature LGL and the NK activity increased. The cytochemical study of the leukaemic cells revealed an enzymatical peculiarity: besides an enzymatic profile characteristic for relatively mature LGL, the cells also displayed peroxidase activity. This unusual aspect--the expression by an individual malignant cell of markers believed to be restricted to a single cellular lineage--might be interpreted as a lineage infidelity or lineage promiscuity. The observation that this lymphoma developed in a patient with a long history of hypogammaglobulinaemia is of particular interest.
Subject(s)
Leukemia, Lymphocytic, Chronic, B-Cell/complications , Leukemia/etiology , Adult , Agammaglobulinemia/complications , Antibodies, Monoclonal , Antigens, Surface , Cytotoxicity Tests, Immunologic , Humans , Leukemia/immunology , Leukemia/pathology , Leukemia, Lymphocytic, Chronic, B-Cell/immunology , Leukemia, Lymphocytic, Chronic, B-Cell/pathology , MaleSubject(s)
Leukemia, Myeloid, Acute/diagnosis , Adolescent , Adult , Aged , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Female , Hemostasis , Histocytochemistry , Humans , Karyotyping , Leukemia, Myeloid, Acute/blood , Leukemia, Myeloid, Acute/drug therapy , Leukocyte Count , Leukocytes/metabolism , Male , Middle AgedSubject(s)
Granulocytes/pathology , Monocytes/pathology , Myelodysplastic Syndromes/diagnosis , Adolescent , Adult , Aged , Bone Marrow/pathology , Cells, Cultured , Colony-Forming Units Assay , Evaluation Studies as Topic , Female , Humans , Male , Middle Aged , Myelodysplastic Syndromes/mortality , Myelodysplastic Syndromes/pathology , PrognosisSubject(s)
Leukemia, Myeloid/etiology , Myeloproliferative Disorders/complications , Adult , Antineoplastic Agents/therapeutic use , Cell Transformation, Neoplastic , Chronic Disease , Female , Humans , Male , Polycythemia Vera/complications , Primary Myelofibrosis/complications , Thrombocythemia, Essential/complicationsSubject(s)
Leukemia, Myeloid/therapy , Splenectomy , Humans , Postoperative Complications , PrognosisSubject(s)
Leukemia/etiology , Polycythemia Vera/complications , Primary Myelofibrosis/etiology , Adult , Female , Humans , Male , Middle AgedSubject(s)
Hodgkin Disease/therapy , Laparotomy , Neoplasm Staging , Splenectomy , Adolescent , Adult , Child , Female , Hodgkin Disease/pathology , Humans , Liver/pathology , Lymph Nodes/pathology , Male , Middle Aged , Spleen/pathologySubject(s)
Purpura, Thrombocytopenic/therapy , Splenectomy , Adolescent , Adult , Aged , Chronic Disease , Female , Humans , Male , Middle Aged , Postoperative Care , Postoperative Complications , Prednisone/therapeutic use , RecurrenceSubject(s)
Polycythemia Vera/therapy , Community Health Centers , Humans , Polycythemia/diagnosis , RomaniaABSTRACT
The course of two cases of severe aplastic anemia (AA) was followed up in two young male patients who, besides the conventional therapy, received perfusions with blood obtained from patients with polycythemia vera (PV). Case 1 received 4 units of PV blood from a unique donor. During the following week an obvious clinical relief, as well as increase of the peripheral blood values were recorded. Subsequently, the bone marrow was progressively repopulated with normal precursors and the peripheral blood picture reverted to normal. In case 2, 9 units of PV blood, obtained from two donors, were administered. During the next week a transient clinical and hematological improvement was obtained, but later on the progressive granulocytopenia and the infections led to exitus through a toxic-septic shock. It is assumed that PV blood may be a good replacing and a possible stimulatory therapeutic material, useful to overcome the early critical stage of severe AA.