Clin. Radiol. (1977) 28, 545-548

A B D O M I N A L L Y M P H O G R A P H Y IN T H A L A S S A E M I A M A J O R

COLIN PARSONS

From the Department o f Radiology, T.P.M.R.A.F. Hospital, .A krotiri, Cyprus*

The appearances are described of abdominal tymphography in seven patients with thalassaemia major. All were severely and chronically anaemic. An abnormal course of lymphatic channels was seen in only one case. Nodal filling defects were found in all patients, lying centrally and peripherally, in para-aortic and iliac glands. The findings correlate well with the microscopic features of lymph nodes removed at therapeutic splenectomy. These show haemosiderin deposition and foci of extramedullary erythropoeisis.

/~-Thalassaemia major is a genetically determined haemoglobinopathy, in which there is selective de- pression in the rate of /3-globin chain production. Clinically there results a severe anaemia which be- comes apparent within the first year or two of life. The condition was first described in patient's whose ancestors derived from the Mediterranean littoral but is now found world wide.

Mediterranean anaemia represents the largest simple genetic disorder in the world. (Weatherall, 1972). Thalassaemia trait occurs with the same high incidence amongst Cypriots in London as it does in Cyprus, about 14%. Between 1961 and 1965 inclusive 25 children with thalassaemia major were born in the boroughs of Camden, Islington and Harringay (Modell et al., 1972).

Surgeons undertaking therapeutic splenectomy in thalassaemia major are familiar with enlarged purple abdominal lymph nodes. Microscopy shows these nodes to contain haemosiderin and erythropoetic foci (Weatherall and Clegg).

There appears to be no previous report of the lymphographic appearances in this condition.

MATERIAL AND METHODS

A 15 year old boy with thalassaemia major was referred for radiological investigation of a retro- peritoneal mass obstructing the common bile duct. He had previously undergone laparotomy, at which an irremovable mass was found behind the common bile duct. A biliary diversion procedure had been carried out, but no biopsy taken.

Considering the nature of the patient's primary disease, bipedal lymphography was undertaken, by the method of Kinmonth (1952) as adapted by Dolan

*Present Address:- P.M.R.A.F. Hospital, Halton, Aytes- bury, Bucks.

and Moore (1962), to implicate lymph node en- largement as the cause of the obstructing mass. Abnormal lymph node appearances and abnormal course of lymphatic channels prompted a small series of lymphograms, to establish the findings to be expected in thalassaemia major. An additional six patients formed the series. Their ages were, 7, 11, 13, 30 and two patients of 31 years.

RADIOLOGICAL FINDINGS

An abnormal course of lymphatic vessels was seen in only one case. This was the first patient in- vestigated. Lymphatics passed normally through the pelvis and abdomen to the level of the second lumbar vertebra where a number passed forward to opacify abnormally sited lymphoid tissue (Fig. l a). This was noted to lie adjacent to the air filled duodenal cap (Fig. lb) and was thought to represent the mass known to be obstructing the common bile duct.

In the further six patients investigated the lym- phatics followed a normal course.

All patients showed nodal filling defects (Fig. 2). The para-aortic nodes were affected more markedly than iliac nodes, but in all the lymphograms both regions were involved to some extent. The rounded lucent areas within the contrast filled nodes lay both centrally and peripherally. When a number of defects occurred in a single node they remained discrete entities, each defect retaining its rounded outline. Multiple defects were often seen in contact with each other. The lucent areas were 1-5 mm in diameter.

In some nodes normal lymphoid tissue was re- placed to such a degree that contrast medium occupied only a peripheral crescent. Subjectively there was a gradual change from coarsely granular nodes to those with multiple measurable translucent falling defects (Fig. 3). The abnormal appearances were least apparent in the youngest patient and most marked in the adults.

546 C L I N I C A L R A D I O L O G Y

Fig. 1 (a) Oblique view of abdominal l ymphogram in a thalassaemic patient showing contrast med ium extending forward at the level of L2. (b) AP view of the same case showing contrast medium related to the displaced duodenal cap.

There was no para-aortic or iliac lymph node enlargement. The largest abdominal node opacified is shown to the left of IA in Fig. 3, this measured 2 8 m m x 15 mm on the AP film, within the normal limits of size found by Jackson and Kinmonth (1974).

DISCUSSION

Increased density of lymph nodes has been noted on plain films of the abdomen in thalassaemia major (Winchester et al., 1973). This increase in density is due to haemosiderin deposition. The enormous body load of iron which accumulates in this condition is stored largely in the reticuloendothelial tissues. Iron accumulates as a result of haemolysis, increased iron absorption from the bowel and from blood trans- fusion. Hardisty (1972) estimates that most patients

receive 4 - 8 g of iron per year from blood trans- fusion and excrete 3 - 4 g of this with the help of a chelating agent, desferrioxamine. Extramedullary erythropoiesis in response to the chronic and severe anaemia is found in the liver, spleen, kidneys and para- vertebral tissue, it also occurs in the lymph nodes.

The abnormal lymphographic appearances in this small series were least apparent in the youngest patient and grossest in the adults and represent a response not only to the severity of the anaemia but also to its duration.

One may anticipate that the degree of lym- phographic abnormality will be related to the success of blood transfusion in maintaining the haemoglobin level, so decreasing the extramedullary erythropoietic effort, and to the success of chelating agents in preventing haemosiderosis.

A B D O M I N A L L Y M P H O G R A P H Y IN T H A L A S S A E M I A M A J O R 547

Fig. 2 - Lymphogram of adult thalassaemic patient showing multiple filling defects throughout abdominal nodes. Characteristic bony changes of the disease are present.

Fig. 3 - Lymphogram of a thalassaemic child shows nodes with many minute lucencies.

No cause has been demonstrated for the abnormal course of the contrast medium in the first patient investigated. A biopsy here would have been helpful. One should certainly consider that extramedullary haematopoiet ic tissue was responsible.

Many pathological processes cause nodal ap- pearances similar to those described here. Metastatic malignant disease and lymphomatous conditions are the most common, but tuberculosis, sarcoidosis, multiple myeloma (Viamonte et aL, 1963), Whipple's disease (Gold and Margolin, 1971), amyloidosis (Davidson and Officer 1969) and Waldenstrom's macroglobulinaemia (Whitehouse et al., 1974) have all shown similar nodal defects.

The clinical features of thalassaemia major make the diagnosis obvious; indeed the patient is unlikely to survive long enough to produce the lymphographic changes unless the diagnosis has been made and treatment started.

A difficulty may arise in the assessment of the lymphogram in a thalassaemic patient who in addit ion has metastat ic disease or a lymphoma. In that case full use should be made of follow up films at intervals to show the progress, or response to therapy, of the second disease.

Acknowledgement. - I am indebted to the Director General of Medical Services, Royal Air Force for permission to publish this paper.

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Dolan, P. A. & Moore, A. B. (1962). Improved technique of lymphangiography. The American Journal of Roent- genology. 88, 110-111.

548 CLINICAL RADIOLOGY

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