Biomedical subjects
Publications and source records attributed to U E Klein.
A large family with sinus node dysfunction is described, spanning three generations and with an autosomal dominant trait. There was an obvious association between the grade of mental retardation and the severity of the sinoatrial disorder.
An acute life endangering bradycardy-tachycardy-syndrome in a 37 years old female could be referred to a rare familiar sinuatrial and atrioventricular arrhythmia with an inherited autosomal dominant trait. The patient's mother, three brothers, one sister and four children showed arrhythmias ranging from sinusbradycardia to second degree heart block. There was an obvious coincidence between intellectual retardation and the extent of the cardiac arrhythmia. Familial arrhythmias should be considered in instances of sudden death or rhythm disorders in young individuals.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Observations in six adult patients with leukaemic differential white counts, predominantly mature-celled, and with hepatosplenomegaly show that the mature-celled but fulminant (para-)neutrophil leukaemia must be differentiated from Ph1-positive chronic myeloid leukaemia. This (para-)neutrophil leukaemia is probably identical with the previously described atypical chronic myelosis of the adult, chronic myeloid leukaemia of childhood and the Pelger-like chronic myeloid leukaemia. Cardinal signs are a mature-celled differential count, short life expectancy (1 year), initial platelet deficiency, increased activity of granulocyte alkaline phosphatase, absence of Ph1-chromosome, and poor therapeutic response to busulfan. This curious and yet apparently not uncommon disease has been observed in the adult age group predominantly in men. The frequently high HbF level observed in juvenile chronic myeloid leukaemia could not be demonstrated in adults. Some of these neutrophil leukaemias are characterized by medullary fibrosis and terminal increase of immature blast cells (blast crises?) of which the diagnostic reliability is still disputed.
Chronic myeloid leukemia without the Philadelphia chromosome (Ph1-CML) is described and distinguished from chronic myeloid leukemia with the Philadelphia chromosome (Ph1+CML) on the basis of clinical and autopsy findings of four cases. Ph1-CML showed clinical, hematological, and patho-anatomical features which could be regarded as typical. Patho-anatomically Ph1-CML differed from Ph1+CML in the variable maturation of the leukemic proliferation in the bone marrow and extramedullary infiltrates. Up to the terminal phase Ph1-CML can be of an extremely mature cell type. However, it can also show myeloblastic transformation after an initially mature cell stage. Ph1-CML infiltrates are found in tissues and organs which Ph1+CML usually does not infiltrate or only to a low degree until a blastic crisis. On the basis of its course and clinical and patho-anatomical features Ph1-CML looks like an atypical chronic myeloid leukemia. However, it is better called an acute myeloid leukemia of the mature cell type.
In clinical hematology the terms "monocytic" leukemia and "reticulosis" still -require better definition and classification. By presenting the histories of eight patients and by cytology and cytochemistry it is shown that myelo-monocytic leukemias can have the course well-known for an acute leukemia, including different skin lesions, as well as that of an typical chronic granulocytic leukemia. In dermatology monocytic leukemias were considered as belonging to the entity of the so-called reticulosarcomatosis cutis. However, strict differentiation from the hiary cell leukemia is to be made today. The general term "reticuloses" has quite faulty been used formerly for classification of these last two disorders.
Explore the source record for details and available documents.