Severe skin rash in two consecutive patients treated with 2-chlorodeoxyadenosine for hairy cell leukaemia at a single institution.

Although hairy cell leukaemia was first described 40 years ago, it is only in the last decade that newer therapeutic agents have enabled effective treatment. The purine nucleoside analogue, 2-chlorodeoxyadenosine (2-CdA) is currently considered as first-line therapy with a very high rate of complete remission. Although adverse events with 2-CdA are increasingly recognized, severe cutaneous reactions have been reported rarely. We describe two consecutive patients treated with 2-CdA for hairy cell leukaemia who both suffered extremely severe cutaneous reactions, one of which was life-threatening.

Infection in immunocompromised patients with malignant blood disorders in a district general hospital.

The pattern and management of infection in immunocompromised patients over a period of 3 years in a district general hospital has been studied. A total of 222 positive cultures was obtained in 607 episodes of suspected infection all involving patients with malignant blood disorders. Febrile episodes requiring intravenous antibiotics occurred in 248 instances involving 107 patients. The pattern of organisms cultured and the responses to various antibiotic regimes are reported. The costs of antibiotic therapy are considered in the light of the overall response. Of the patients studied, 54 died, infection having a likely causative or contributory part in 21 of them (less than 10% of infective episodes). We conclude that the infective complications of these disorders, particularly in older patients, can be efficiently managed in a district general hospital when full supportive facilities are available.

Acute promyelocytic leukaemia and Hodgkin's disease in the same patient.

Acute non-lymphocytic leukaemia is a well recognised and increasingly reported complication of treated Hodgkin's disease. The prognosis is generally poor with a disappointing response to chemotherapy. A patient in whom myelodysplastic features appeared after treatment for Hodgkin's disease, to be followed shortly afterwards by acute promyelocytic leukaemia, is reported. Complete remission was achieved and sustained until Hodgkin's disease reappeared three years later when the patient was autografted with a marrow harvested four years earlier. The patient remains in good health with platelet support at the time of writing.

Two alien hand syndromes.

Review of the clinical characteristics and neuroanatomy of 20 reported cases of alien hand syndrome (AHS) and a patient of our own confirm that AHS is actually two distinct syndromes. Frontal AHS occurs in the dominant hand; is associated with reflexive grasping, groping, and compulsive manipulation of tools; and results from damage to the supplementary motor area, anterior cingulate gyrus, and medial prefrontal cortex of the dominant hemisphere and anterior corpus callosum. Callosal AHS is characterized primarily by intermanual conflict and requires only an anterior callosal lesion. the occurrence of frontal AHS in the dominant limb can be explained by an increased tendency for dominant limb exploratory reflexes coupled with release from an asymmetrically distributed, predominant nondominant-hemisphere inhibition. Callosal AHS is best explained by hemispheric disconnection manifested during behaviors requiring dominant-hemisphere control.

Mitozantrone and cytosine arabinoside as first-line therapy in elderly patients with acute myeloid leukaemia.

We have prospectively evaluated a regimen of mitozantrone and cytosine arabinoside (Ara-C) as first-line therapy in elderly patients with acute myeloid leukaemia (AML). One hundred and four patients with a median age of 68 (range 60-81) were studied, in whom 86 had de-novo AML, and 18 had preceding myelodysplasia or secondary AML. Complete remission was achieved in 64% of de-novo cases, in 28% of MDS/secondary cases, and in 58% overall. The incidence of early death within 28 d of chemotherapy was 11%. The median disease-free survival (DFS) was 11 months with an actuarial DFS of 15% at 43 months. The median overall survival was 9 months with an actuarial survival of 10% at 44 months. The incidence of non-haematological toxicity was acceptably low, and usually of mild to moderate severity. Quality of life was improved, or unchanged, in 90% of responders. We conclude that mitozantrone and ara-C is an effective and well-tolerated regimen which produces high remission rates in elderly patients with AML.

Infectious mononucleosis with acute thrombocytopenia.

Three cases of severe acute thrombocytopenia complicating infectious mononucleosis are described. Although spontaneous remission may be a feature of this rare complication, in each of these cases steroid therapy resulted in a rapid rise in the platelet count.

Lactic dehydrogenase estimation in haematological malignancies.

Earlier studies have indicated a role for serum lactic dehydrogenase (LDH) in haematological and other malignancies. As this is still not a routine test in many units, its value has been re-examined in a retrospective analysis of serum LDH levels in 278 patients with haematological malignancies managed in a single unit. Abnormal levels at diagnosis were found in 42% of these disorders; yet analysis of over 23,000 routine requests for a biochemical screen showed only 4% with an abnormal LDH level. The estimation was also valuable in follow-up of those haematological patients, with rising levels often indicating progressive or relapsing disease even in cases which had shown a normal LDH at diagnosis.

Problems of myeloma in a community.

The presenting features of 120 consecutive cases of myeloma diagnosed in a single unit were examined. Screening tests on routine laboratory samples increased the detection rate but did not significantly reduce the percentage of cases presenting with late stage disease. Morbidity was closely related to the incidence of bone involvement already present at diagnosis. About one fifth of cases had reported suspicious symptoms for some considerable time before further investigation was forthcoming. Almost half had 'benefitted' by having concurrent disease which often led to the discovery of myeloma at an early stage. These findings, together with the wide variety of symptomatology and few physical signs, stressed the critical importance of having a high index of suspicion and thus taking suitable blood samples without unnecessary delay.

The anion gap as a screening procedure for occult myeloma in the elderly.

Myeloma in the elderly can mimic many disorders, causing delay in diagnosis. In a retrospective study, the mean anion gap at presentation in 78 elderly patients with confirmed myeloma was found to be significantly less than that of aged-matched controls. It is suggested that estimation of the anion gap may be useful as a screening test, particularly as it has no intrinsic cost.

HLA-A,B and DR antigens in chronic lymphocytic leukaemia.

Eighty-three patients with B cell chronic lymphocytic leukaemia were typed for HLA-A,B, and DR antigens. HLA-A2 was found to be significantly increased in patients compared to controls. The frequency of antigens A2,B12(44),DR7 occurring together was more than double the frequency found in the controls. In 48 patients requiring treatment, there was a significant reduction of B8 when compared to 34 untreated patients, and DR7 was significantly increased in frequency in treated patients. Thirty patients presented with sub-clinical symptoms (Rai stage 0). HLA-A10 was significantly more common than in those patients presenting with more advanced disease (Rai stages 1-4). The highest frequency of any HLA antigen was found in males presenting with clinical symptoms, 71.0 per cent of whom were A2 positive; in the same group, 22.6 per cent of patients were positive for all the antigens A2,B12(44),DR7. Patients with an age of onset greater than the mean age of 69 years were more likely to be A2 positive. We conclude that HLA-B8 is a marker of mild disease while HLA-A2 and the closely associated antigens B12(44) and DR7 are markers for severe disease.

Extramedullary transformation of chronic myeloid leukaemia with a unique chromosome pattern.

A case of extramedullary myeloblastic transformation of chronic myeloid leukaemia is reported. This was characterised by an unusual clinical and haematological course and showed a unique cytogenetic pattern.

Biological studies with continuous-wave radiofrequency (28 MHz) radiation.

Effects of high-frequency (28 MHz) continuous-wave radiation have been studied in the rat and monkey. No histopathological (rat-125 mW cm-2 for 28 days) or hematological (monkey-25 mW cm-2 for 24 days) changes could be attributed to the radiation. In the monkey (125 mW cm-2 for 11 days) there was an increase in urinary calcium concentration which was most likely due to restricted movement. In the rat (220 mW cm-2 for 13 days) there was reduced uptake of iodine by the thyroid, lower levels of plasma thyroid-stimulating hormone, and reduced ratio of protein bound to nonprotein bound iodine. Food consumption was also decreased. The changes are likely to have arisen as a compensatory response to an induced heat load. A nonthermal effect of continuous-wave high-frequency radiation has not been shown in this study. The effects were likely to be associated with either physiological compensation for induced heating or restriction of movement.