Janos Radó:
My “in between” hematological studies during a 66-year medical career as a nephro-endocrinologist
Although I am basically a nephrologist (nephro-endocrinologist), during my 66 yearlong activities as an investigator-clinician I have dealt with some hematological topics.
1. Pelger-Huet Anomaly of the Leukocytes.
2. Successful Extraction of Complicated Cataract in a Patient Suffering from Haemophilia B.
3. Mononucleosis Infectiosa.
4. Steroid Ecchymosis.
5. Microangiopathic Hemolysis.
1. Pelger-Huet Anomaly of the Leukocytes
The first Hungarian family with Pelger-Huet anomaly of leukocytes has been described. Each member of the family was completely complaint free and no other abnormality could be detected, with the exception of one member (see below).
Hammer S, Radó J. Pelger-Huet anomaly of leukocytes. Orvosi Hetilap 97 pp. 298-301. 4 p. (1956) (in Hungarian).
In one member of the family with Pelger-Huet anomaly of the leukocytes, polycythemia vera was turning into myelofibrosis. The clinical evidence tends to indicate that in man the heterozygous anomaly is an innocuous variation: (a) the white blood cell changed in this case, as is common in cases of polycythemia not associated with the anomaly, (b) the myeloid system producing the anomalous cells showed no increased sensitivity to cytostatic effects; and (c) it appears that notwithstanding the occurrence of numerous complications the leukocytes fulfilled their physiologic protective functions.
Radó JP, Hammer S. Polycythemia vera turning into myelofibrosis in an individual with Pelger-Huet anomaly of the leukocytes. Blood 14 pp. 1143-1150. 8 p. (1959).
2. Successful Extraction of Complicated Cataract in a Patient Suffering from Haemophilia B
A hemophilic male patient 52 years of age had had numerous gastrointestinal bleedings since the age of 36. The family history contained no episode of hemophilia. The results of the thromboplastin generation test were consistent with the diagnosis of Christmas disease (hemophilia B). Successful extraction of an uveitic complicated cataract was performed under the cover of transfusion and dexamethasone. We suppose our case is worthy of publication because of its rarity and usefulness of the steroid treatment.
Borbely L, Radó JP, Tako J, Farkas G, Szabados D. Studies on the effect of adrenal cortex hormones on hemophilia. Folia Haematologica 87:358-372 (1967).
de Grosz I, Borbely L, Szabados D, Radó JP. Successful extraction of complicated cataract in a patient suffering from haemophilia B. Acta Ophthalmologica 43: 574-578. 5 p. (1965).
Grosz I, Borbély L, Szabados D, Radó J. Sikeres hályogkivonás B-tipusu haemophiliában szenvedő betegen. Szemészet 102: 76-80, (1965) (in Hungarian).
3. Mononucleosis Infectiosa
One hundred and thirty-five cases with mononucleosis infectiosa have been investigated. Two types of leukocytosis were found: I. Early leukocytosis, within the first 5 days of the disease, consisting high number of normal granulocytes, and II. Late leukocytosis, occurring after the first 5 days of the disease (generally between day 10 and 16) consisting of mononuclear cells.
Devenyi P, Radó J. Mononucleosis infectiosa Orvosi Hetilap 95 464-469. (1954) (in Hungarian).
4. Steroid Ecchymosis
Role of various factors in the pathogenesis of skin hemorrhage (steroid ecchymosis) due to glucocorticoids.
Radó J, Hamvas J, Bikich G. Role of various factors in the pathogenesis of skin hemorrhage (steroid ecchymosis) due to new glucocorticoids. Magyar Belorvosi Archivum 14 pp. 107-118, 12 p. (1961) (in Hungarian).
5. Microangiopathic Hemolysis
Authors refresh and describe a patient suffering from thrombotic microangiopathy induced by gastric cancer who was observed under their care 54 years ago. With the help of literature review they analyze the development of clinical studies and available treatment options during the past half century. This review contains also medical historical issues of the age. The 47-year-old patient who underwent four years earlier a Billroth II gastric surgery because of a duodenal ulcer disease, presented with the symptoms of a hematological disorder, hemolytic anemia, fragmentocytosis, leukoerythroblastosis and thrombopenia.
The clinical picture resembled that of the seven patients who were observed by the coauthor from 1955 till 1962 and who were presented in 1963 at a Hematological Conference held in Pécs, Hungary. The author’s tentative clinical diagnosis was metastatic gastric cancer, though there was no conclusive evidence to confirm it. The illness became fatal with an unusually rapid progress. Histology proved the presence of metastatic gastric cancer, though macroscopically no tumor could be found at necropsy.
Conclusion is the importance of the hematologic alterations in the detection of hidden metastatic (gastric) cancer. The knowledge of the syndrome of microangiopathic hemolysis, fragmentocytosis, leukoerythroblastosis, thrombopenia associated with metastatic cancer is helping to recognize the cause of the disease among the numerous origins of thrombotic microangiopathy. It is suggested that there is a strong relationship between the “big hemolysis” and the tumorous obstruction of the pulmonary arterial tree.
Szende L, Radó J. Microangiopathic Haemolysis and Leukoerythroblastosis in Carcinomatosis of Gastric Origine. Case Report, Review of the Literature, Medical History. MBA Magyar Belorvosi Arcivum 2021. No 1.
Summary
1. Pelger-Huet Anomaly. The first Hungarian family with Pelger-Huet anomaly of leukocytes has been described. In one member of the family with Pelger-Huet anomaly of the leukocytes polycythemia vera was turning into myelofibrosis. The white blood cell changed in this case, as is common in cases of polycythemia not associated with the anomaly and the myeloid system producing the anomalous cells showed no increased sensitivity to cytostatic effects.
2. Successful Extraction of Complicated Cataract in a Patient Suffering from Haemophilia B. The results of the thromboplastin generation test were consistent with the diagnosis of Christmas disease (hemophilia B). Successful extraction of an uveitic complicated cataract was performed under the cover of transfusion and dexamethasone.
3. Mononucleosis Infectiosa. One hundred and thirty-five cases with mononucleosis infectiosa have been investigated. Two types of leukocytosis were found.
4. Steroid Ecchymosis. Role of various factors in the pathogenesis of skin hemorrhage (steroid ecchymosis) due to new glucocorticoids.
5. Microangiopathic Hemolysis. The knowledge of the syndrome of microangiopathic hemolysis, fragmentocytosis, leukoerythroblastosis, thrombopenia associated with metastatic cancer is helping to recognize the cause of the disease among the numerous origins of thrombotic microangiopathy. It is suggested that there is a strong relationship between the “big hemolysis” and the tumorous obstruction of the pulmonary arterial tree.
October 21, 2021