52
946 Proc. roy. Soc. Med. Volume 69 December 1976
(Table 1). A peroral jejunal biopsy showed total villous atrophy characteristic of cceliac disease. A gluten-free diet was instituted upon which the patient showed progressive clinical, hmematological and biochemical improvement (see Table 1) with an overall weight gain of 18.6 kg. Repeated jejunal biopsies over the succeeding 25 months Conclusion To summarize, a patient is reported who has (Fig 1) demonstrated a return to normal of the congenital hypoplasia of the inferior vena cava, a histological appearances, reflected by an increase carotid aneurysm and intercurrent infectious in the villous and epithelial surface cell heights. mononucleosis. This confusing collection of December March February conditions was brought to light by a presenting 1976 1973 1975 left and then right deep venous thrombosis of the Meanvillousheight±s.d.(m) 204±38 251 ±46 311±36 33 ±4 Mean epithelial cell 25±2 34±4 legs. height ±s.d. (g,m)
The close anatomical association of the internal carotid artery with the vagus nerve and sympathetic chain suggest that direct pressure of the aneurysm is the most likely cause for the nerve lesions seen in this man.
REFERENCES Ferris B J, Hipona F A, Kahn P C, Philipps P & Shapiro J H (1969) Venography of the Inferior Vena Cava and its Branches. Krieger, Huntington, New York Nicolaides A N ed (1975) Thrombo-embolism, Aetiology, Advances in Prevention and Management. MTP, Lancaster Sprbul E (1938) American Journal of Cancer 34, 566-585 Thomson A P (1938) Clinical Journal 47, 137
Celiac Disease with Hodgkin's Lymphoma J Seggie MB MRCP (for L J Farrow MB MRCP) (Watford General Hospital, Watford,
Hertfordshire, WDJ 7HH) A housewife aged 30 presented in December 1973 with a two-year history of weight loss, flatulence, nausea, intermittent vomiting and diarrhea consisting of up to ten bulky stools a day. She was extremely emaciated, weighing 39 kg. Her tongue was smooth and she appeared anemic. Investigations revealed a picture of malabsorption with evidence of megaloblastic anemia, hypoalbuminmmia and hypocalcimia
This favourable course was marred by the discovery, in March 1975, of enlarged lymph nodes in the left axilla and supraclavicular fossa. Biopsy confirmed nodular sclerosing Hodgkin's disease classified as Stage IIA at laparotomy. The patient was treated with mantle radiotherapy. She remained well until February 1976 when she drew attention to a mass in the left breast which was fixed and associated with left axillary lymphadenopathy. Biopsy of both confirmed recurrence of the Hodgkin's disease which had now become Stage IVA. Accordingly, combination cytotoxic therapy (MOPP) was started. Discussion Gough et al. (1962) first drew attention to the association of reticulum cell sarcoma with adult coeliac disease. Since then, it has become widely accepted that malignancy, be it lymphoma or carcinoma, is a specific complication of celiac disease. Three-quarters of the lymphomata are of the reticulum cell variety, the remainder being largely Hodgkin's disease. Only one-third do not involve the intestinal wall; wholly extra-abdominal disease, as in our patient, is uncommon (Harris et al. 1967, Cooke 1970).
Table 1 H2matological, biochemical and serological improvement following gluten withdrawal December 1973 May 1974
Hb (g/dl)
MCV(Itm')
MCHC (%)
RBCfolate(pg/ml;NR 150-900) Serum iron (,ug/dl; NR 60-200)
Serum iron-binding capacity (tg/dl; NR 200-430) Serum albumin (g/l; NR 3.7-5.3) Calcium (mmol/l; NR 2.20-2.63) Alk. phosphatase(iu/l; NR 50-200) IgA (tg/dl; NR 150-350) Reticulin antibody NR, normal range
8.8 126 32 120 25 270
July 1974 November 1974 March 1975 February 1976
-
-
-
-
-
12.7 92. 32.3 350 -
-
-
-
-
-
36 2.15 135
-
7.9 78 28.8 110
8.1 81 27.8 200
10.1 88 28.7
32 1.96 125 540
39 2.23 175
44 2.20 200
-
-
-
Negative
-
-
-
11.7 87 32
39
360 Negative
44 2.35 97 300
Negative
Clinical Section
53
947
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946 Proc. roy. Soc. Med. Volume 69 December 1976
(Table 1). A peroral jejunal biopsy showed total villous atrophy characteristic of cceliac disease. A gluten-free diet was instituted upon which the patient showed progressive clinical, hmematological and biochemical improvement (see Table 1) with an overall weight gain of 18.6 kg. Repeated jejunal biopsies over the succeeding 25 months Conclusion To summarize, a patient is reported who has (Fig 1) demonstrated a return to normal of the congenital hypoplasia of the inferior vena cava, a histological appearances, reflected by an increase carotid aneurysm and intercurrent infectious in the villous and epithelial surface cell heights. mononucleosis. This confusing collection of December March February conditions was brought to light by a presenting 1976 1973 1975 left and then right deep venous thrombosis of the Meanvillousheight±s.d.(m) 204±38 251 ±46 311±36 33 ±4 Mean epithelial cell 25±2 34±4 legs. height ±s.d. (g,m)
The close anatomical association of the internal carotid artery with the vagus nerve and sympathetic chain suggest that direct pressure of the aneurysm is the most likely cause for the nerve lesions seen in this man.
REFERENCES Ferris B J, Hipona F A, Kahn P C, Philipps P & Shapiro J H (1969) Venography of the Inferior Vena Cava and its Branches. Krieger, Huntington, New York Nicolaides A N ed (1975) Thrombo-embolism, Aetiology, Advances in Prevention and Management. MTP, Lancaster Sprbul E (1938) American Journal of Cancer 34, 566-585 Thomson A P (1938) Clinical Journal 47, 137
Celiac Disease with Hodgkin's Lymphoma J Seggie MB MRCP (for L J Farrow MB MRCP) (Watford General Hospital, Watford,
Hertfordshire, WDJ 7HH) A housewife aged 30 presented in December 1973 with a two-year history of weight loss, flatulence, nausea, intermittent vomiting and diarrhea consisting of up to ten bulky stools a day. She was extremely emaciated, weighing 39 kg. Her tongue was smooth and she appeared anemic. Investigations revealed a picture of malabsorption with evidence of megaloblastic anemia, hypoalbuminmmia and hypocalcimia
This favourable course was marred by the discovery, in March 1975, of enlarged lymph nodes in the left axilla and supraclavicular fossa. Biopsy confirmed nodular sclerosing Hodgkin's disease classified as Stage IIA at laparotomy. The patient was treated with mantle radiotherapy. She remained well until February 1976 when she drew attention to a mass in the left breast which was fixed and associated with left axillary lymphadenopathy. Biopsy of both confirmed recurrence of the Hodgkin's disease which had now become Stage IVA. Accordingly, combination cytotoxic therapy (MOPP) was started. Discussion Gough et al. (1962) first drew attention to the association of reticulum cell sarcoma with adult coeliac disease. Since then, it has become widely accepted that malignancy, be it lymphoma or carcinoma, is a specific complication of celiac disease. Three-quarters of the lymphomata are of the reticulum cell variety, the remainder being largely Hodgkin's disease. Only one-third do not involve the intestinal wall; wholly extra-abdominal disease, as in our patient, is uncommon (Harris et al. 1967, Cooke 1970).
Table 1 H2matological, biochemical and serological improvement following gluten withdrawal December 1973 May 1974
Hb (g/dl)
MCV(Itm')
MCHC (%)
RBCfolate(pg/ml;NR 150-900) Serum iron (,ug/dl; NR 60-200)
Serum iron-binding capacity (tg/dl; NR 200-430) Serum albumin (g/l; NR 3.7-5.3) Calcium (mmol/l; NR 2.20-2.63) Alk. phosphatase(iu/l; NR 50-200) IgA (tg/dl; NR 150-350) Reticulin antibody NR, normal range
8.8 126 32 120 25 270
July 1974 November 1974 March 1975 February 1976
-
-
-
-
-
12.7 92. 32.3 350 -
-
-
-
-
-
36 2.15 135
-
7.9 78 28.8 110
8.1 81 27.8 200
10.1 88 28.7
32 1.96 125 540
39 2.23 175
44 2.20 200
-
-
-
Negative
-
-
-
11.7 87 32
39
360 Negative
44 2.35 97 300
Negative
Clinical Section
53
947
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