Yukihiro Kawaguchi

ULTRASTRUCTURAL STUDY ON THE RELEASE REACTION OF THE PLATELET

The ultrastructural changes of the platelets stimulated by release inducers II such as thrombin, collagen, and latex particles were studied to compare the differences in morphology of release I and II. The characteristics of platelets in response to thrombin were the decrease in number of alpha‐granules and marked dilation of the open canalicular system containing fibrin preceded by the disappearance of very dense granules. In the platelets in response to collagen or latex, the release of alpha‐granules was initiated after taking the particles into the open canalicular system. In all of the cases, the amount of microfibrils was seen in the cytoplasm, while contraction wave of the microtubules was rare in the cytoplasm during the release and uptake of particles. Accordingly, the release of very dense granules seems to be followed by that of alpha‐granules. It is suggested that the microfibrils play a more important role than the microtubules in the release II as well as release I. Furthermore, the contents of alpha‐granules may be discharged via the open canalicular system on the release II by thrombin, collagen or latex particles.

Widespread eczema vaccinatum acquired by contacts. A report of an autopsy case.

A4‐moth‐old male infant predisposed to allergic dermatitis acquired widespread eczema vaccinatum by contacts with a recently vaccinated sibling. He died of acute purulent peritonitis following a perforation of multiple duodenal ulcers. Fluorescence immunocytochemical and electron microscopic studies on the skin lesions revealed the presence of viral antigens and numerous virus particles compatible morphologically with those of the mature form from the same batch of smallpox vaccine given to the sibling. A large number of virus particles in the developmental form were also predominantly scattered in the cytoplasm of cells at the stratum malpighii of the epidermis as well as in neutrophils and macrophages in the skin lesions. The virus isolation from the skin lesions was done by using the HeLa cells and the human embryonic lung fibroblasts. No abnormal laboratory data were noted in immunoglobulins. On the basis of atrophy of the thymus and other lymphatic tissues and an appearance of large pyroninophilic cells in association with blastoid transformation, the authors discussed a possible participation of the disturbance of cellular immunity secondary to the virus infection in the development of the disease. ACTA PATH. JAP. 29: 435–455, 1979.

Type iii glycogenosis with deposition of urate and amyloid

A case of a 44‐year‐old man with hepatic form of glycogenosis was presented. The patient had abdominal distension and muscular weakness. The glucose tolerance test showed a diabetic pattern, though he had hypoglycemia in fasting state. The fructose tolerance test showed an ability of conversion from fructose to glucose. The double glucagon test showed no rise of blood glucose in fasting state but a rise 2 hours after meal. These symptoms and laboratory data supported the clinical diagnosis of type III glycogenosis. At autopsy, glycogen was markedly deposited in the liver, and slightly in the kidneys and heart. The glycogen pooled in the hepatic cells histochemically showed a normal reaction to several glycogen stainings. Electron microscopy by using Thiérys method revealed that the pooled glycogen particles were clearly arranged as rosettes measuring 1.000Å in largest diameter composed of clustered monoparticulates. There were marked hyalinization of the islets of Langerhans containing amyloid. As to its pathogenesis, this change can be interpreted as a morphological expression of the hypofunction of β‐cells ascribed to long‐standing hypoglycemia.

ANOMALOUS ORIGIN OF THE LEFT CORONARY ARTERY

Two autopsy cases of anomalous coronry artery were reported. The patients were a schoolgirl and a schoolboy, who died suddenly during physical exercise at the age of twelve. At postmortem examination in both cases the left coronary artery arose at the comissure from aorta, and in one case the left coronary ostium was completely obstructed with fibrous tissue, whereas in the other case it was severely stenotic. The cause of anomalous ostial position should be regarded as congenital, but the complete occlusion and severe stenosis at the orifice seemed to be modified by several factors ‐ both congenital acquired. For the prevention of sudden death caused by this anomaly, cardiac angiography would have been necessary at the outset of anginal pain.

HISTIOCYTIC MEDULLARY RETICULOSIS

An autopsy case of histiocytic medullary reticulosis (HMR) in a 55‐year‐old man is presented. The initial complaint of the patient was an unusual skin lesion on the right upper arm, and subsequently developed generalized erythematous skin lesions which regressed during the course of the illness. The clinical symptoms and course of the disease were those of HMR. The affected organs were lymph nodes, spleen, liver and bone marrow as well as the skin, in which erythrophagocytic histiocytes predominated in abnormal cells of the histiocytic series. Phagocytic histiocytes had large amounts of cholesterol and small amounts of phospholipids which might be originated from the ingested erythrocytes. The electron microscopy suggested the presence of phospholipid‐cholesterol interaction in the phagocytic histiocytes.

FAMILIAL MEDULLARY CARCINOMA OF THE THYROID THROUGH 3 GENERATIONS

A family with high genetic penetrance of medullary carcinoma of the thyroid was reported. Seven proven (6 patients and one autopsy case) and 2 probable cases of medullary carcinoma were present in 25 members through 3 generations. An endogamy had intervened in the prior generation of these cases. Preliminary results in clinicopathological examinations of 7 proven cases were as follows; clinically, all of the cases showed B type blood group. Glycosuria was found in 2 cases, and diarrhea in one case. Serum thyrocalcitonin being estimated In two cases showed high levels. The autopsy cases coexisted with medullary carcinoma of the thyroid and pheo‐chromocytoma of the right adrenal. Pathologically, the majority of tumors occurred in both thyroid lobes, and were present from the middle to upper portion of the thyroid. The tumor showed a variety of histological features even in the same tumor. In the tumor cell, numerous membrane‐limited granules were seen with an electron microscope. Amyloid was demonstrated only In the tumor tissue.