Search

A 70-year-old man was admitted to Jichi Medical School Hospital on October 1, 1990, complaining of spontaneous severe hemorrhage in his extremities. He had prolonged APTT value and normal PT value. Factor VIII level was markedly diminished, and a high titer of factor VIII inhibitor was observed. He was diagnosed as having idiopathic factor VIII inhibitor because of no sign of underlying disorders. He received prednisolone and immunosuppressants, subsequently with plasma exchange and bolous factor VIII, resulting in disappearance of the inhibitor. He was admitted again in February 1991, because of the reappearance of factor VIII inhibitor. In June, furthermore, bone marrow smears showed infiltration of atypical B cells, although no evidence of organomegaly and adenopathy was observed. Therefore, he was suspected to have primary bone marrow lymphoma. He received CHOP therapy, but he died of multiorgan failure. Postmortem autopsy demonstrated multifocal intravascular lymphomatosis with marked intravascular involvement of the lung, liver, kidney, adrenal glands, spleen and bone marrow. Lymph nodes were not swollen, showing little evidence of intravascular lymphomatosis.

Neoplastic angioendotheliosis (NAE) is a rare condition characterized by proliferation of atypical cells in small vessels of various organs. In the past, atypical cells seen in this condition were considered to originate from vascular endothelial cells. However, recent immunohistochemical studies have established that the tumor cells in this condition mainly originate from B-lymphocytes. NAE is likely to affect the central nervous system and skin. Cases of NAE with a primary lesion in the pulmonary vessels are rare. One such rare case of NAE was recently encountered by us. The patient was a 77-year-old woman who was admitted to our department because of fever and interstitial shadow in both lungs. After admission, fever did not subside, and the pulmonary shadow became more marked. Laboratory tests upon admission disclosed elevated LDH. Of isozymes, LDH2 and LDH3 were high. Anemia and thrombocytopenia gradually became aggravated, and atypical cells with large basophilic cell body and irregularly shaped nucleus subsequently appeared in peripheral blood. Thereafter, the patient's general condition worsened rapidly and she died about 5 months after admission. Post-mortem examination revealed pleomorphic atypical cells filling the lumen of small vessels in various regions of the body. This finding was particularly marked in pulmonary vessels. With various immunohistochemical stains, these cells were identified as cells of B-lymphocyte origin.

We measured immunoreactive endothelin-1 in plasma of a patient with malignant hemangioendothelioma. A 74-year-old female first visited our clinic in July, 1989. Physical examination revealed reddish nodule measured 4 x 5 x 1 cm and erythematous plaque with erosion on her scalp. A biopsy specimen showed extensive proliferation of atypical tumor cells intermingled with a large number of capillary vessels in dermis. Some of tumor cells were positively stained for factor VIII related antigen by PAP method. From these findings we diagnosed malignant hemangioendothelioma and resected the tumor on August 3. The measurement of immunoreactive endothelin-1 in plasma was performed by radio-immunoassay on August 2 (before operation), October 20 and December 27 (after operation). The results were 16.2, 4.3 and 5.6 pg/ml, respectively (normal range: 0.5 +/- 0.2 pg/ml). Plasma endothelin-1 level may be run parallel to quantity of tumor cells in malignant hemangioendothelioma.