Rituxan in the treatment of cold agglutinin disease.
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Aged↗
Biomedical subjects
B Kueck
Publications and source records attributed to B Kueck.
Acute leukemia and the transient myeloproliferative disorder associated with Down syndrome: morphologic, immunophenotypic and cytogenetic manifestations.
Individuals with Down syndrome have an increased incidence of leukemia compared to the general population. In addition, Down syndrome children may acquire a myeloproliferation that resembles acute leukemia that undergoes a spontaneous, durable remission. To clarify the relationship between these two disorders, the morphologic, immunophenotypic and cytogenetic characteristics of 28 patients with Down syndrome and the morphologic manifestations of acute leukemia were examined. Three cytomorphological groups were discerned. The first two groups consisted of five patients with acute lymphoblastic leukemia (group I) and three patients with acute myeloid leukemia (group II). These leukemias resembled those of non-Down individuals. The third and largest group (group III) consisted of 20 cases of acute myeloid leukemia that showed prominent megakaryocytic and/or erythroid differentiation and occurred in children under 6 years of age. The blasts in this group were non-reactive for myeloperoxidase or non-specific esterase and expressed CD7, CD34 and CD36 with variable expression of CD61, CD13 and CD33. Four patients in this group had an acquired trisomy 8. Four group III leukemias underwent a durable, spontaneous remission within 2 months of diagnosis. There were no morphologic differences between those leukemias in this group that progressed and those that remitted; however, all remissions occurred in newborns. It is concluded that Down syndrome children acquire a characteristic acute myeloid leukemia that has prominent megakaryocytic and/or erythroid differentiation and an unusual immunophenotype. This group of leukemias may undergo a durable, spontaneous remission in the newborn period.
Acute Disease↗
S100-positive, T-cell chronic lymphoproliferative disease: an aggressive disorder of an uncommon T-cell subset.
S100-positive T lymphocytes account for less than 3% of peripheral blood T cells. Rare cases of S100-positive T-cell lymphoma have been previously described. We report four such cases of S100-positive T-cell chronic lymphoproliferative disease. In all cases, hepatosplenomegaly was observed, without prominent lymphadenopathy. Central nervous system (CNS) involvement by the leukemic cells was suggested in three cases by physical symptoms and confirmed in two cases by cerebrospinal fluid studies. Despite treatment, three patients died at 3, 6, and 8 months after diagnosis. Although there was a leukemic presentation, only minimal bone marrow infiltration was evident. Splenectomy showed red pulp infiltration. Liver and lymph node biopsies showed sinusoidal leukemic involvement. In all cases, the leukemic cells expressed mature T-cell- and natural killer cell-associated antigens. Cytoplasmic S100 was detected in the leukemic cells in the blood, spleen, liver, and lymph node. Southern blot studies in two cases showed T-beta, T-gamma, and T-delta gene rearrangements. RNA Northern blots showed T-alpha and T-beta chain transcripts with no T-gamma or T-delta RNA identified. Southern blot analysis showed no hybridization to probes specific for Epstein-Barr virus, cytomegalovirus, human immunodeficiency virus-1, or human T-cell lymphotropic virus type-1. These findings show that S100-positive T-cell chronic lymphoproliferative disorder is an aggressive, extramedullary-based disease frequently associated with CNS involvement and characterized by short survivals.
Adult↗
Systemic polyclonal immunoblastic proliferations.
This report describes the clinical and pathologic features of four patients with a florid, systemic immunoblastic proliferation. The blood of these patients exhibited a mild to marked leukocytosis with a high percentage of immunoblasts and plasma cells. The bone marrow also was infiltrated extensively by immunoblasts. Lymph node biopsy specimens from two patients showed near total effacement of the nodal architecture by a diffuse infiltration of immunoblasts and plasma cells. The proliferative process was determined to be polyclonal with immunohistochemical techniques. Cytogenetic studies of bone marrow from two patients showed a pseudodiploid abnormal clone, with a translocation involving a break in band 14q32 in each case. The pathogenesis of these proliferative disorders in unclear, although three patients had some evidence of an acute immune disorder. One of these patients was treated with steroids, vincristine, and cyclophosphamide. Another patient was treated with steroids only, and one patient was treated with steroids and cyclophosphamide. All had rapid regression of the disease process. Two patients are alive and apparently free of disease 31 and 48 months after diagnosis. One died of sepsis. The fourth patient had acquired immune deficiency syndrome (AIDS) and died without therapy. The biology of the immunoblastic proliferation of these patients is uncertain. The immunohistochemical results suggest a reactive, polyclonal proliferation, but the cytogenetic abnormalities in two patients indicate the possibility of a cryptic neoplastic clone.
Acquired Immunodeficiency Syndrome↗
Argyrophilic, 'carcinoid-like' prostatic carcinoma. An immunocytochemical study.
Four cases of argyrophilic or carcinoid-like prostatic carcinoma were studied immunocytochemically, using immunoperoxidase stains for prostatic-specific antigen, neuron-specific enolase, hydroxytryptamine (serotonin), somatostatin, and adrenocorticotropic hormone. All four showed strong positive reaction to prostatic-specific antigen and uniformly negative results with neuron-specific enolase, hydroxytryptamine, somatostatin, and adrenocorticotropic hormone. These findings lend further support to the concept that this particular prostatic tumor is truly a carcinoma that somehow manifests a carcinoid-like histomorphology, but does not possess evidence of true neuroendocrine or carcinoidal nature.
Adenocarcinoma↗