Complications and. Treatment of Acquired Aplastic Anaemia

Abstract

SUMMARY The clinical and haematological findings in 43 cases of aplastic anaemia are reviewed. Approximately two‐thirds of the cases were considered to be secondary to toxic agents, the commonest of which were chloramphenicol and phenylbutazone, and one‐third were idiopathic. Severe neutropenia and thrombocytopenia were a feature of the series, a neutrophil count of less than 500/cu.mm. and a platelet count of less than 20,000/cu.mm. being found initially in over one‐half of cases. There was an unexplained and marked elevation of the ESR in over 90 per cent of cases. Fifty‐two episodes of infection occurred in 29 patients (67 per cent); 36 of these episodes were serious. Infection was more frequent in young patients and serious infection was more likely in the presence of severe neutropenia. The occurrence of infection, especially serious infection, was usually followed by a fall in neutrophil and platelet counts, and frequently by the onset of serious bleeding. Bleeding phenomena occurred at some stage in all but one patient and in two‐thirds of cases the bleeding was serious. Thrombocytopenia was marked in patients with serious bleeding but equally low platelet counts were found in patients in whom bleeding was not serious. The onset of serious bleeding was more likely in the presence of infection. Administration of corticosteroids or corticotrophin either with or without androgens was followed by a haematological response in about two‐thirds of cases, but in only one‐third was the response sustained; in the remainder it was transient. In those with a sustained response, the haemoglobin and neutrophil values gradually increased and ultimately returned to normal; however, the platelet count returned to normal in less than one‐half of these. The overall mortality rate was 63 per cent, bleeding and infection being the commonest causes of death. The mortality was higher in the idiopathic cases (86 per cent) than in the secondary cases (52 per cent); however, the period of survival in fatal secondary cases was shorter than that in fatal idiopathic cases. The prognosis appeared to be better in patients with secondary aplasia, in patients with an initial haemoglobin value greater than 8 g. per 100 ml., and in patients who remained free of serious bleeding. The outcome showed no relation to sex, age, the initial neutrophil and platelet counts or the cellularity of the bone marrow on admission. Seven of the 16 survivors are considered cured, eight are well but are still thrombo‐cytopenic, and one has developed paroxysmal nocturnal haemoglobinuria 8 years after diagnosis and 6 years after a complete haematological remission. Treatment with corticosteroids is still necessary to control bleeding in three of the eight survivors with thrombocytopenia.

Treatment of Aplastic Anaemia with Methenolone

“…The sequence of the remission signs in our series was similar to that described in earlier reports [5,10]. The recovery of the platelet count was most delayed and is still incomplete in many cases.…”

“…The introduction of testosterone in the treatment of aplastic anaemia in children [9] was followed by less successful trials in adult patients [4,8,10]. Later on, anabolic steroids with less androgenic activity were used in place of testosterone and good results were achieved in both children [1,3,5,6] and adults [2,5,6,11].…”

Acquired Aplastic Anaemia in Adults

“…In other patients corticosteroid therapy in either high or low dosages was not followed by dramatic im provement. This is in agreement with results of other authors [15,26,27,50]. The addition of corticosteroids to androgens only aggravates the vir ilizing effects according to Gardner [11], who suggested that steroid therapy is only indicated in case of marked haemolysis.…”

“…It has been shown that manifest infection can also in crease the incidence of severe haemorrhage [54], Splenectomy led to definite improvement in 1 patient out of 7 in our series. The absence of a direct influence on the haematological status in this case confirms the findings of V incent and de Cruchy [50], G ross et al [15], and van der W eijd en and F irkin [52], On the other hand, sple nectomy does improve the efficiency of transfusions. The platelet recov ery after transfusion in particular is increased in these patients.…”

Oxymetholone Treatment in Hypoproliferative Anaemia¹

“…Later studies revealed that im provement occurred in only a few adults [5,9,13,16]. In 1964, Sanchez-Medal et al [11] reported remission in 70% of cases of various types of chronic refractory anaemia after treatment with a new anabolic steroid, oxymetholone.…”