Haemangiosarcomatosis: Two Cases Presenting as Haematological Problems

Abstract

Circumcision-Barrie et al. rDImNA 275The attitude of the medical profession in this country against circumcision has hardened in recent years, so much so that sincere and reasonable requests by the parents are sometimes turned down, which may cause distress and result in the mother canvassing other doctors or hospitals in search of a willing surgeon. The parents' desire for their baby to be circumcised may be based on firmly held beliefs concerning hygiene, national or religious convictions, or a desire for conformity among the males of the family. Our policy in such cases is to perform the operation if the parents still request it after a frank discussion of the medical viewpoint. SummaryA technique for circumcising the newborn, using a plastic bell to protect the glans, which also acts as a guide to the amount of prepuce to be removed, is described. Case 1A 33-year-old housewife developed pleuritic pain in February 1961. A month later she felt tired and ill, with pains in the limbs, and had become pale and very dyspnoeic. On admission to a local hospital in May she was febrile and the tip of the spleen was just palpable. A moderate normochromic anaemia was present (Hb 7.1 g./100 ml.) with a small number of red-cell and white-cell precursors in the peripheral blood. The total white-cell count and platelet count were normal. All investigations, including blood urea, liverfunction tests, blood culture, and a search for L.E. cells were negative. No definite diagnosis was reached.Prednisolone, penicillin, and blood transfusions were given. She improved and was discharged home on a reduced steroid dosage. Five days later, however, she was readmitted with epistaxis, purpura, vomiting, and pain in the left arm. The haemoglobin level had fallen to 4.9 -g./100 ml. Steroid therapy was continued and she was transfused repeatedly. Four weeks later, when she was transferred to the Royal Infirmary, Manchester, there was still extensive purpura. In spite of the blood she had received the haemoglobin was still only 7.6 g./100 ml. The platelet count had fallen to 40,000/c.mm., and the leucocytes numbered 18,000/c.mm. There was still a leucoerythroblastic peripheral blood picture.All investigations remained negative. A sternal marrow specimen was unhelpful. However, a radiological skeletal survey revealed multiple osteolytic foci indicating widespread tumour involvement. She continued to receive steroids and blood transfusions ; in addition, intravenous nitrogen mustard therapy-2 x 6 mg. Trillekamin (trimustine hydrochloride)-was given. There was no response; her condition deteriorated and 14 days later she died.Pathology.-Necropsy confirmed the presence of haemorrhagic and partly necrotic tumour tissue in the upper end of the femora, humeri (with a pathological fracture through a deposit in the proximal left humerus), thoracic vertebrae, and throughout the rib cage. Tumour deposits were also present in the liver, spleen, right ovary, and right atrium. The lymph nodes were not involved.None of these tumour deposits could be conf...

Haemangiosarcoma of the petrous temporal bone

“…Haematological complications have been seen, the most frequent being microangiopathic haemolytic anaemia (Brain et al, 1962;Ludwig and Hoffman, 1975). A case of leukoerythroblastic anaemia due to marrow metastasis has been reported (Bourne et al, 1965).…”

Microangiopathic haemolytic anaemia associated with malignant haemangio-endothelioma

“…Bourne, Cook, and Williams (1965) have described two patients with a leuco-erythroblastic anaemia which they attributed to infiltration of the marrow by haemangio-endothelioma. None of these workers remarked on a haemolytic element of the anaemia.…”