84 THE INDIAN MEDICAL GAZETTE [Feb., 1939

ATYPICAL AMYLOID DISEASE OF THE LIVER

By M. D. ANANTHACHARI, m.b., b.s.

(General Hospital, Madras)

With the disappearance of instances of long- continued suppuration and chronic destructive

disease, cases showing amyloid degeneration have become exceedingly rare. Only one case is recorded among the autopsies at the General

Hospital, Madras, during the past twenty-five years. When cases are detected on the autopsy table, unsuspected during life, it becomes evident

that association with chronic suppuration and tissue destruction are not the invariable antece- dents of amyloid degeneration. It is stated that amyloid disease may be found in association with malaria after long-continued infection or re-

peated attacks (Gibson, 1936; Boyd, 1938). Such post-mortem revelation has resulted in the necessity to suspect amyloid disease, even though no apparent basis for this diagnosis is noted (Bannick et al., 1933). It is possible that the condition escapes recognition because it is not suspected. Bennhold (1923), working with the dye Congo

red as a stain for amyloid tissue, pointed out that intravenous injection of this dye would dis- close the presence of amyloid disease during life* As a result of this report Congo red has been extensively used in suspected cases of amyloid disease and ante-mortem diagnosis, has become possible. Tests both quantitative and qualita- tive are described and their value as clinical aids to diagnosis appraised. Through this test it has also been made possible to recognize that the piocess of amyloid degeneration is reversible and that improvement is possible in cases where the

?oooa Can be removed (Wallace, 1932; Walker, ly^o).

AA aldenstrom practised a system of bio- puncture suggested by Arnold' Josefson. He diagnosed the disease by examining the material withdrawn by puncturing the liver with a hyp0' dermic needle. Of interest is his experience that, even in a patient undoubtedly suffering from ainyloid disease, the hepatic cells aspirated by a nne needle are always healthy, unless a pieee as large as a liver acinus (about 1.5 mm. dia- meter and 2 cm. in length) is removed. He con- cludes that a probability from mere clinical

tion1106 becomes a certainty by such examina- Depending as it does on the mass of existing

amyloid tissue in the body, the Congo-red test las its limitations. It is not possible that the test will reveal early cases. Bennhold observed tnat patients ̂

with extensive amyloidosis, espe' cially, amyloidosis of the liver, practically all gave positive Congo-red tests and that it was on y when the deposits of amyloid were not extensive that one might obtain a negative bongo-red test. In the case under report this statement is probably borne out.

.

b it Possible that amyloid disease could arise pnmarily without any discoverable cause era and Gross (1935) raise a point whether

some previous suppurative process might not initiate a disturbance in protein metabolism, w nch would survive the initial perverse impetus- ?/ (1938) mentions a case where there was

extensive amyloidosis without any discoverable caube at autopsy. Only very few cases appea1' *? u j']e^0r.^e(^ where there was no cause. Might it be that in such cases diet produces a disturb- ance which hitherto was conceived to be initiated only by tissue destruction ? Experimental evidence suggests that diet, particularly cheese,

Feb-> 1939] ATYPICAL AMYLOID DISEASE OF LIVER : ANANTHACHAR1 85

has its share of influence in the setiology of

disease. It is also not known what it is tliax

determines the site of such changes or how it

is

that in certain cases the amyloid change is

i

fused and in certain others it is localized as

tumour mass.

. C'ase record.?A man, aged 35 years,

was

lntp the wards of the General Hospital, Madras,

Pain, and enlargement of the abdomen. A labourer, he was a poorly nourished

man wi

Protuberant abdomen which lie complained was

gradually becoming harder and bigger. He had p

round the waist and in the epigastric region, paitic-

ularly after food though his appetite _

was good, ne

was not constipated. There was nothing abnormal m

his diet. ?n palpation of the abdomen the liver was felt

as a hard mass extending across the epigastrium ? ,

left hypochondrium. It was firm, smooth and pamle-s

and had a rounded edge. In the left hypochond" felt.slightly harder in consistency than

elsewhere

x?ere it simulated very closely in all clinical

an enlarged spleen. For diagnostic purpose the mass

!n the left hypochondrium was punctured with a nyp

dermic needle of fairly large bore. The aspira

material was a clear jelly-like substance of tne

consistency of boiled sago, without any trace

of blooa

in it. ^he material was sent to the pathologist report is appended. , ,, .

Screening the stomach with barium showed

that it

was normal in shape and movements. It appeared

Pushed forward and to the right. .

e

ihere was neither ansemia nor jaundice, His

was normal. The van den Bergh test was negative

aud the icterus index 7 units. Blood cholesterol was

J79.4 mgm. per cent. The aldehyde and Chopra te^ts

tor kala-azar were negative. Blood serum for llahn s

test was negative. f

^9 eliminate the possibility of a latent f?cus, ?

sepsis, white blood cell counts were repeatedly done

and the maximum count was only 9,900 cells per c.mm.

Arneth count showed no shift to the left. Sedimenta-

tion rate, according to Zechwer and Goodall, was

r-4 cm. jn one hour Functional test for liver with

Xylose was normal. , , ,

io verify the histological findings Congo-red test=

ere carried out according to methods described y

Taran (1937) and Todd and Sanford (1935). Both the

0Sri? gave normal healthy figures. ihe patient according to his statement felt better

ana at the end of two months gained eight pounds in

height. He left hospital at his own request and

against advice, with the liver in the same condition.

,\athologist's report.?Smears stained with metny

violet show small masses of metachromatically (pmk)

gaining, wavy, branching macaroni-like bands of a

homogeneous substance, with fine bluish-staming fibrils

separating the individual strands. Occasionally, scat-

hed between these masses of pink-staining cylindeis

? v ?lusters of large polyhedral cells (staining blue;

which in some p]aces appear compressed and

el?ngated. A few collections of degenerate leucocytes

\vV a> seen- The endothelial lining 9f the capillaries

nich can be clearly distinguished in some places

dPPears to be free from Leishman-Donovan bodies,

/ne general appearance is not unlike that, of a

smear

a\ an. amyloid liver in which extensive amyloid

eposition has led to marked disappearance of the

Parenchyma cells.

Comment

The outstanding points in this case are the

Absence of any clinically discoverable cause for

tlle condition;' the difference between the two

[^arts of the enlarged liver, the failure of the

^?ngo-red tests, and the normal function of the

lyer. Autopsy elucidation was however absent

That none of the known causes for amyloid disease is present cannot be asserted in this case. There is however no evidence of chronic sepsis or suppuration. Might it be that the causes for the enlargement for the right and left halves of the liver are different, the left lobe alone being affected by amyloid disease ? Rosenblum and Kirshbaum (1936) in classifying amyloidosis mention a possible localized involvement with

amyloid, which may occur within neoplasms or chronic inflammatory areas. Such would explain the failure of the Congo-red tests on the basis of insufficiency of amyloid material to give a positive test. The absence of ascites, jaundice, the good functional condition of the liver, in spite of its size, are points of interest.

In this connection, it is to be noted that Paunz test is associated with certain practical difficul- ties. The amount of Congo red to be injected is bulky. It is sometimes found difficult to draw blood at the end of one hour, as the rapid coagulation of the blood in the needle interferes with aspiration. A similar observation is made by Wallace (1932) and Becker. The test is not delicate and it is useless in early cases. Its only virtue is that it can be carried out in places where colorimetry is not possible.

Wallace (1932) claims that the Congo-red method possesses obvious advantages over

Waldenstrom's bio-puncture. According to him it gives a quantitative result and is somewhat less drastic. In the case reported, without the puncture the disease would certainly have been missed. Where suspicion of the disease is strong from clinical evidence and requires only confir- mation the Congo-red method will be the choice. In other cases where the clinical history does not lead to suspicion at all or where the disease is very early, bio-puncture alone can supply un- equivocal evidence. The method of bio-puncture is not however available where kidney alone is

the site of amyloid disease and where the liver and spleen are not enlarged for safe puncture. Where the kidney is affected early, the disease is more readily recognized by examination of the urine and the associated clinical evidence of

poor kidney function. It is in cases in which

the liver or the spleen alone is involved primarily that, if a Congo-red test, which is not infallible, fails, the disease will be altogether missed.

Summary 1. A case of amyloid disease of the liver with

unusual features is reported. 2. The usefulness of the Congo-red test and

bio-puncture is discussed.

Acknowledgment I am grateful to the superintendent, General

Hospital, Madras, for permission to publish this case.

Bibliography

Bannick, E. G., Berkman, J. M., and Beaver, D. C. (1933). Arch. Intern. Med., Vol. LI, p. 978.

(Continued at foot of next page)

0Continued from previous page)

Bennhold, H. (1922). Miinchner vied. Woch., Vol. LXIX, p. 1537. (Abstract?Med. Sci. Abst. and

Rev., 1923, Vol. VIII, p. 59.)

Bennhold, H. (1923). Arch. Klin. Med., Vol. CXLII, p. 32.

Boyd, W. (1938). Textbook of Pathology. Lea and

Febiger, Philadelphia. Gibson, A. G. (1936). British Encyclopaedia oj

Medicine. Vol. I, p. 401. Butterworth and Co., Ltd., London.

Perla, D., and Gross, H. (1935). Amer. Joum. Path., Vol. XI, p. 93.

Rosenblum, A. H., and Kirshbaum, J. D. (1936). Joum. Amer. Med. Assoc., Vol. CVI, p. 988.

Taran, A. (1937). Joum. Lab. and Clin. Med., Vol. XXII, p. 975.

Todd, J. C., and Sanford, A. H. (1935). Clinical

Diagnosis by Laboratory Methods. W. B. Saunders

Co., Philadelphia. Walker, G. F. (1928). Lancet, Vol. II, p. 120.

Wallace, J. E. (1932). Ibid., Vol. I, p. 391.