ANGINA AGRANULOCYTICA AND ITS TREATMENT.

This form of sepsis, which has been much studied during recent years, is accompanied by a striking diminution of the white cells in the blood. The total leucocyte count may fall to 300 per c.mm., the

granular cells?the polymorphs in particular?being almost entirely absent. The clinical picture is characterised by severe gangrenous inflammatory processes affecting particularly the throat and buccal

mucous membrane, high fever, extreme leucopenia and often an ill-

marked secondary ansemia. In most cases the disease is rapidly fatal. The condition was first described by Shultz in 1922 and since then numerous other clinicians both in Germany and America have con- firmed and extended his observations. Hamburger (1), in discussing the fifteen cases which have come under his observation at Johns Hopkins Hospital, points out that the title angina agranulocytica is best confined to those cases in which the etiological agent is unknown and which

do not include other well-recognised conditions in which a similar state of the blood is commonly found. The localisation of the lesions in the mouth and throat make it desirable that other leucopenic states, such as aleuksemic, leuksemic, aplastic ansemia and arsenical and benzol

poisonings, be classed in a separate group, as they are distinct from the present condition, which is apparently a peculiar form of septicsemia running an acute and often fatal course.

Of the 15 cases described in this paper, 14 were in women, 11 being forty years of age and over. All complained of sore throat and had oral lesions varying from follicular tonsillitis to necrosis

involving the fauces or oral mucosa. The clinical picture in the

early stages was not unlike influenza?accompanied by great prostration and general aching, with sore throat. Three had cutaneous gangrene. One had jaundice. The primary leucocyte counts averaged 900 per c.mm., the average percentage of polymorphs was 9, with a minimum

figure of o and an initial maximum of 28. Certain clinical types may be differentiated. There is a fulminating

variety, death following in a day or two, often with great respiratory 208

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Periscope embarrassment. A second group have a more prolonged illness, either to die or recover in a few weeks, and these cases present enlargement of the cervical glands or the brawny induration of Ludwig's angina. Other cases have recurrent attacks and often die in a relapse. A

chronic form has recently been reported by Thayer and his associates (2), Jn a man of 20 years of age who had recurrent attacks of stomatitis with agranulocytosis, fever and constitutional symptoms at intervals of

approximately three weeks throughout his life. , Many organisms have been isolated from the mouths and throats

of these patients. Vincent's organisms are frequently found and various micro-organisms have been recovered from the blood. They are all

probably secondary invaders. Just as the etiological agent is unknown, so is the nature of the defect in the leucopoietic system totally obscure.

Many methods of treatment have been employed, mostly with the intention of making good the obvious defect in the blood picture. It is not at present easy to assess the efficacy of any line of treatment in a disease which runs such a varying course, particularly as severe cases may recover spontaneously. Repeated transfusions have been used but the reports are conflicting. Salts of guanine and adenine, to stimulate polymorphonuclear production, have been used, and this method seems worthy of further trial before final conclusions can be drawn. Local treatment consists of spraying with a saturated solution of potassium chlorate, followed by swabbing of the ulcerated areas and gums with copper sulphate, 10 grains to the ounce. More recently it has been suggested to employ X-ray therapy as a means of

stimulating the production of polymorphonuclear leucocytes?on the principle that small doses stimulate, large destroy. Waters and Firor (3), have treated five patients with repeated exposures of the long bones to 1/20 of an erythema dose. Four of these patients recovered.

While an improvement in the general condition and also in the blood has been noted in patients following this line of therapy, these authors do not quote any figures to show the effect of the rays on the production of white cells. They perhaps wisely confine their conclusion to the

guarded impression that a trial of X-ray therapy is at least justifiable for these unfortunate patients.

(1) Hamburger, L. P., "Angina Agranulocytica and its Treat-

ment" {Bull. Johns Hopkins Hosp., 1931, xlviii., 339). (2) Rutledge, B. H., Hansen-Pruss, O. C., and Thayer, W. S. {Bull. Johns Hopkvis Hosp., 1931, xlvi., 369). (3) Waters, C. A., and Firor, W. B., "Roentgenotherapy of Angina Agranulocytica" {Bull. Johns Hopkins Hosp., 1931, xlviii., 349). A. R. G.

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