HISTIOCYTIC MEDULLARY RETICULOSIS

650

HISTIOCYTIC MEDULLARY RETICULOSIS A CASE WITHOUT LYMPHADENOPATHY

RICHARD ASHER Lond., M.R.C.P.

’

M.B. PHYSICIAN,

CENTRAL MIDDLESEX COUNTY HOSPITAL

UNDER the name

of histiocytic medullary retioulosis characterised by fever, wasting, generalgroup of ised lymphadenopathy, and enlarged liver and spleen, and sometimes, in the final stages, by jaundice, purpura, anaeinia, and leucopenia were collected by Scott and Robb-Smithy The case reported here closely resembles them, except that there was no lymphadenopathy and the enlargement of the spleen was more rapid. There was a superficial resemblance to some of the a

node the size of a cherry in the left anterior triangle of the no other nodes were felt. Nothing else abnormal There was no family was found on physical examination. history of blood disease and no history of contact with chemicals or medicines. Her blood-count on admission showed a severe anaemia., Hb 49%, colour-index 0-87, red cells 2,870,000 per e.mm., with many macrocytes (average diameter 8µ), and a leucopenia of 2500 white cells per c.mm. with a relative lympho. cytosis (86%). The platelet-count was normal (200,000 per c.mm.). Reticulocytes were 1%. Fragility of the red cells

neck, but

cases

’

idiopathic hsemolytic anaemias, but against them were the normal fragility, the low reticulocyte-count, and. the unraised van den Bergh. Correlation of the amounts transfused with changes in blood-count also revealed abnormal blood destruction. Myelosclerosis was ruled out by the absence of radiographic bone changes and

no

the absence of a leuco-erythroblastic blood-picture. The progress after splenectomy is another point against this diagnosis, for in Ihyelosolerosis splenectomy is usually fatal. Chronic splenic neutropenia2 bears some resemblance to this case but shows hyperplasia of the myeloid cells in the bone-marrow, blood reticulosis, and a raised serum-bilirubin level.3 Moreover, sections of the spleen gave an entirely different picture. CASE-RECORD

On Oct. 19, 1944, a shorthand-typist, aged 35, was admitted to the Central Middlesex County Hospital with three months’ history of growing paler and getting short of breath. For three days she had felt weak and feverish and had an aching pain in the loins, with painful frequent micturition. On examination she had a striking pallor, showing an earthy yellow tinge rather like that of leuksemia. The liver and spleen were enlarged (see fig. la). The enlargement was nrm, smooth, and not painful on pressure. There was a soft lymph 1. Scott, R. B., Robb-Smith, A. H. T. Lancet, 2. See Ann. intern. Med. 1945, 22, 271. 3. Wiseman, B. K., Doan, C. A. Ibid, 1942,

1939, ii, 194. 16, 1097.

Fig. 2-Patient’s chart showing

course

Fig. I-Diagrams illustrating enlarsement Nov. 20 ; (c) Dec.

of spleen : 20.

(a)

Oct. 20 ;

(b)

Sternal puncture revealed a highly cellular normal. with a dearth of white cells at all their stages. There was no free hydrochloric acid in the alcohol histamine testmeal. The was 103° F. A few colonies of Bact. coli were grown from a catheter specimen of urine ; but five days later, and again on Nov. 11, 1944, no organisms were detected in catheter specimens and no growth developed on culture. For the next nine weeks she ran a high temperature, her tvhitecell count diminished steadily, and the spleen rapidly enlarged. Medical Treutment.-No treatment, except blood-transfusions, had any effect.’Fersolate ’ gr. 12 t.d.s. was given throughout her illness. Liver extract (’Anahæmin’ 2 c.cm.) produced no reticulocyte response, nor did proteolysed liver (’ Hepamino ’). , A course of’Pentnucleotide’ (260 c.cm. given intravenously and intramuscularly in fourteen days} provoked no increase in white cells. ’ Sulphamezathine,’ totalling 15 g., and later penicillin 435,000 units were given empirically to combat any vulnerable infection, but the fever did not abate, nor did the blood-picture change. The patient steadily deteriorated, the white-cell count fell rapidly to 200 per c.mm. on Dec. 13, 1944. The spleen enlarged so rapidly that the edge advanced more than in. in twenty-four was

marrow,

of illness and

temperature

immrldiate

improvement on splenectomy.

651 .

hours. It

was

were made

on

carefully marked out on several dates (fig. 1).

Splenectomy.-After

nine weeks in

the

skin, and drawingss

hospital

there

was no

doubt that without further interference the patient

So splenectomy was considered justifiable,’’ result was doubtful and the risk great. 0 A week before operation her white-cell count was only 200 her she had and Hb c.mm. though 50%, already y per g received ten transfusions of 540 c.cm. each. She was therefore d given a further 2500 c.cm. of blood during the next week and r went to the theatre with a count of red cells 5,000,000 per e e.mm., Hb 74%, and white cells 2000 per c.mm. During the operation she was given another 1620 c.cm. of blood butt received no more transfusions thereafter. Altogether in the .0 course of her illness she was given thirteen transfusions Le totalling more than 9000 c.cm. of blood. At the operation, performed by Mr. T. G. I. James on J. Dec. 20, 1944, the spleen was found to be bigger than the e clinical examination indicated. The lower pole filled the e left iliac fossa and extended well into that of the right side. 3. The upper pole had elevated the left dome of the diaphragm a considerably. The liver extended 9 cm. below the right costal margin. Its colour and texture were normal. The spleen after removal weighed 1180 g. and measured 30 X 15 X 11c.cm. Progress.-Her condition was fair at the end of this extensive operation, and she improved steadily from that day. Fig. 2 shows the sudden fall of temperature and proliferation of white cells, which were as dramatic as they were unexpected. Within twenty-four hours the temperature was normal and remained so, and the white-cell count was already doubled. These changes were accompanied by a corresponding improvement in general health and a rapid gain in weight. Eleven days after the operation she started getting up, and within a fortnight she was walking well. Her white-cell count rose to 16,500 per c.mm. and then settled at an average figure of 3000 per c.mm. She remained afebrile and was discharged from hospital on Jan. 15, 1945, three weeks after the operation. Path,ology.-Sections have been reported on by Dr. W. Pagel, of this hospital, and Dr. A. H. T. Robb-Smith, of Oxford. Both describe the same pathological changes : massive infarction of the whole organ, and between the infarcted areas an excess of cells, the cells being mostly erythrocytes, but among them (1) large phagocytic cells (histiocytes) containing engulfed red cells, and (2) large nonphagocytic cells (prohistiocytes) with single or multiple deeply basophil nuclei. Progress after Discharge.-For four months after discharge she remained well with no symptoms. On May 7 she was readmitted with a recurrence of her old symptoms : pallor, breathlessness, and general weakness. The only abnormal physical signs were a soft enlarged liver reaching 7 em. below the costal margin and a moderate amount of ascites. She A blood-count was running a temperature of 101°—104° F. showed Hb 50%, colour-index 1-1, white cells 1000 per c.mm. (44% neutrophils). Her condition was unchanged for a month, after which she improved spontaneously. The fever subsided, and the blood improved (Hb 90%, white cells 3000 per c.mm.). The liver became smaller, and the ascites disappeared. She was discharged on July 29. Three months later she was admitted with Sonne dysentery. This cleared withSulphasuxidine ’ but she continued to have fever and general weakness for the next two months. Her blood-counts remained relatively normal, showing a mild leucopenia (3000 per c.mm.) and a mild hyperchromic anaemia (lIb 75%). After two months the temperature subsided and the patient was allowed up. She was extremely weak in the and complained of numbness round the mouth and loss legs of taste and smell. Examination showed no objective changes in facial sensation, taste, or smell. She was allowed home at her own request on Nov. 17. Dr. W. T. Hunt, her general practitioner, attended her from then till her death two months later. He has kindly given me details of her final illness. She remained afebrile and of good colour but became progressively weaker and emaciated. She developed increasing weakness of the face and jaw, with severe difficulty in speaking, eating, or swallowing. ’Prostigmin’ had no effect on this weakness (the description slightly resembled that of myasthenia gravis). Three days before death she became jaundiced, had abdominal pain, and mass

of the

spleen.

18

1946.

Unfortunately

SUMMARY

was s

becoming increasingly wasted andd going to die. She was the . anaemia and leucopenia to bloodhelpless, the response of transfusions was only transient, and she was becoming g exhausted by the protracted fever and increasing ponderouss

although the

became comatose. She died on Jan. a necropsy was not performed.

A

of fever,

wasting, enlarged liver and spleen, anaemia, and leucopenia, closely resembling histiocytic medullary reticulosis, except for the absence of lymphadenopathy, is described in a woman aged 35. After splenectomy there was an immediate fall in temperature and a proliferation of leucocytes, accom. panied by such an improvement in general health and weight that the patient was discharged from hospital three weeks after operation. Four months after discharge from hospital she was readmitted with a requrrence of symptoms, but this improved spontaneously. She died at home eight months later; death was preceded by jaundice, abdominal pain, and coma. No necropsy was performed. case

I wish to acknowledge the help of Dr. A. H. T. Robb-Smith, Dr. Avery Jones, and Dr. W. Pagel, and to thank Mr. T. G. I. James for managing such a hazardous splenectomy. Sister Mileham is responsible for the chart.

PYLORIC OBSTRUCTION BY A MASS OF ASPHALT

E. R. DAVIES F.R.C.S. SURGEON,

ANGLO-IRANIAN OIL CO.

LTD.,

ABADAN

AN Iranian labourer, aged 21, had four days’ intermittent upper abdominal pain and vomiting. There was no previous history of abdominal pain, nor did he admit that he had eaten anything abnormal. Vomiting was copious after a meal, but he did not vomit if he took only fluids. On .EamMMMt.—A fit young man of no great intelligence, was not dehydrated and had a normal temperature and pulse-rate. An oval tumour could be felt lying transversely he

in

the e

right hypochondrium and suffi-

ciently mobile

to

pushed

be up

into the epi-

gastrium. A tentative diagnosis of a

pedunculated gastric adenoma

made, Mass of asphalt which obstructed the pylorus the tumour (scale in inches). being oval and too mobile for a subperitoneal hydatid cyst, and the blood-count was normal and Casoni test negative. Owing to a temporary breakdown it was impossible to give a barium meal. Operation.-On laparotomy the tumour was found to be inside the stomach. Incision of the anterior wall revealed a hard black mass, which was delivered without difficulty. The gastric mucosa appeared normal, and the prepyloric portion was normal to’palpation. Patient made a good recovery from the operation. The specimen consisted of a mass of asphalt with some pomegranate seeds embedded in its surface. It weighed 30 g., and its size can be judged from the accompanying photograph. On section it was found to be almost homogeneous, and there were no food particles in the few holes in its substance. Patient later said that he had worked in the asphalt plant about ten months earlier and had been in the habit of chewing small pieces, some of which he swallowed. This habit of chewing asphalt is said to be even more common among workers in asphalt plants in England than it is here. Two more workmen with small abdominal masses have been found out of ten examined. s 2 was

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