The accumulation of amyloid in many organs of patients with multiple myeloma has been well documented, but much less attention has been directed to the presence of amyloid material in joints and in the substance of the skeleton itself. The present report is that of three patients with multiple myeloma in whom the clinical findings of amyloid arthropathy developed. Amyloid infiltration in and about the joints was so extensive as to simulate the findings of rheumatoid arthritis. In two of them, the articular manifestations were present for several months prior to the diagnosis of multiple myeloma. The delay of the diagnosis resulted in the development of a fatal complication of multiple myeloma, renal failure, which was not always unavoidable. Due to the fact that amyloid arthropathy is a rare manifestation of systemic amyloidosis and simulates rheumatoid arthritis, its diagnosis may be easily overlooked.

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The release of goblet cell mucus (GCM) was examined in immune reaction-system of colonic mucosa of normal rats (SD rats). Though there are several studies as for mucus release of small intestine, no papers reporting colonic GCM release are found. Lake A.M. et al. previously reported that in the immune reaction of small intestine of normal rats, the discharge of GCM increased after intraduodenal challenge of specific antigen in the orally immunized animals. We examined release of colonic GCM in antigen-antibody system in rats, who received intrarectal instillation (challenge) of several test-antigens after repeated immunization of single BSA antigen through rectal mucosa. Intrarectal infusion of BSA into immunized rats with BSA produced no greater GCM release than the GCM release obserbed on challenge with buffer alone (control) or other antigen (OVA), comparing with the results in case of small intestine by Lake A.M. et al. These results suggest that there was difference in local antigen-antibody reaction on the surface of normal mucosa between small intestine and colon.

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A 63-year-old woman with a 20-year-history of Takayasu's arteritis was admitted to Keio University Hospital because of fever, oral ulcer, watery diarrhea and erythema nodosum on her legs. Barium enema and endoscopic examination showed multiple erosions in the sigmoid colon. With a tentative diagnosis of incomplete Behçet's disease, administration of prednisolone with an initial daily dose of 30mg was started. The clinical symptoms and laboratory findings showed prompt responses to the steroid therapy. Three additional cases which described coexistence of Takayasu's disease and Behçet's disease have been reported. These patients were characterized by prominent intestinal symptoms and absence or mild ocular lesions. In two patients including present case, HLA typing was done and both had HLA-B 52 and DR 2. A possibility of existence of homogeneous group of patients with unique clinical presentations of Takayasu's disease and Behçet's disease was discussed.

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We report a case of Coombs-negative autoimmune hemolytic anemia which was diagnosed by flowcytometric analysis. A 20 year-old man developed anemia and jaundice following flu-like symptoms in August, 1989. He was admitted to the nearby hospital and diagnosed as hemolytic anemia with unknown etiology. Although he started taking prednisolone 30mg/day, severe hemolysis did not improve. More than five units/day of red blood cells were transfused to maintain the Hb level at 5.0g/dl. He was transferred to our hospital on September 12, 1989. On admissin, he revealed severe icteric skin and marked hepatosplenomegaly. The laboratory data were as follows; Hb 5.4g/dl, reticulocytes 101‰, T. Bil. 10.3mg/dl, D. Bil. 4.0mg/dl, LDH 1, 540 IU/l, haptoglobin<10mg/dl. Bone marrow tap showed marked erythroid hyperplasia. These data were compatible with hemolytic anemia. Although the Coombs' test and Polybrene test were all negative, autoimmune hemolytic anemia was suspected. He was treated with steroid-pulse therapy followed by splenectomy, resulting in complete recovery from the hemolysis. Hemosiderosis caused by massive red blood cell transfusion was effectively treated with repeated depletion of the blood in combination with erythropoietin injection. Anti-red blood cell auto-antibodies were retrospectively identified in the serum before steroid-pulse therapy using flowcytometric analysis. Our method to detect anti-red blood cell antibodies using flowcytometry, which is more sensitive than Coombs' test, would be useful for the diagnosis of Coombs-negative autoimmune hemolytic anemia.

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A 51-year-old woman with pustulosis palmaris et plantaris (PPP) presented with pustulotic arthro-osteitis (PAO). She had arthritis and osteitis in multiple joints and bones. She may have had an allergic tendency, since she was positive for serum antinuclear antibody. There was no increase in WBC, but increased severity of PPP was noted following the tonsil provocation test. Bilateral tonsillectomy was very effective in the treatment of both PPP and PAO. We speculated that both the PPP and PAO were consequences of chronic tonsillitis.

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Here we report a case of systemic lupus erythematosus (SLE) complicated with infection-associated hemophagocytic syndrome which presumably caused by cytomegalovirus (CMV) and pneumonia due to Legionella bozemanii. A 36-year-old woman, who had manifested an occasional rash since 1985, was admitted to a hospital in her hometown in March 1991 because of a sore throat and a high fever, over 39°C. Examinations revealed massive proteinuria, leukopenia, positive anti-nuclear antibody and pleural effusions. A diagnosis of SLE was made and a treatment with 60mg/day prednisolone was initiated followed by a pulse therapy with 1, 000mg/day methylprednisolone for 3 days and then 50mg/day azathio-prine. As the reduction of the dose of prednisolone was found to be difficult, she was transferred to our hospital on October 7, 1991. On admission she was suffering from disseminated intravascular coagulation (DIC) due to reactive hemophagocytic syndrome, whereas there was neither respiratory complaint nor abnormality on the chest X-ray film. Since October 9, the body temperature rose above 38°C. On October 11, she began to cough and abnormal shadow, suggesting pneumonia, was found on the left upper lobe, which rapidly progressed to occupy most of the whole left lung field. The patient, however, was successfully treated with erythromycin and the diagnosis of Legionella bozemanii pneumonia was made because serological studies revealed a significantly elevated antibody suggesting Legionella bozemanii. On the other hand, as cytomegalovirus was isolated from her urine, the administration of ganciclovir was started, and subsequently hemophagocytosis and DIC gradually resolved.
This case is of particular interest in that a concomitant infection of cytomegalovirus, which presumably caused hemophagocytic syndrome, and Legionella bozemanii, one of the rare species causing Legionellosis, which was responsible for pneumonia. It was considered that immunosuppressive state of the patient was important as a background of this case.

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Methylprednisolone (M-PSL) pulse therapy has been sporadically reported in adult patients with idiopathic thrombocytopenic purpura (ITP) who were resistant to a standard treatment with prednisolone. This treatment is considered effective in childhood ITP, but the efficacy is not established in adult ITP.
In this report, we describe four adult patients with ITP who received M-PSL pulse therapy: two patients have maintained a long-term remission or partial remission after M-PSL pulse therapy, and a short-term effect was observed in one patient. The remaining patient, however, did not respond to M-PSL pulse therapy, nor to other treatments such as splenectomy and immunosuppressive agent.
Our results suggest that M-PSL pulse therapy is one of the favorable treatments for adult ITP patients who have a resistant disease to the standard therapy.

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We would like to report a case of thyroid cancer (adenosquamous cell) associated with marked leukocytosis and hypercalcemia. To demonstrate the production of granulocytecolony stimulating factor (G-CSF) secreted from the tumor, the residual tumor tissue was resected under aseptic condition at autopsy and was minced and cultured. High levels of G-CSF in the culture supernate were detected by enzyme linked-immunoadsorbent assay (ELISA) using monoclonal antibodies against human recombinant G-CSF. Therefore, it was evident that neutrophilia in this patient was induced by G-CSF secreted from the thyroid cancer cells. Parathyroid like-hormone which induced hypercalcemia was also suggested to be secreted from the cancer cells in this patient. The cases of thyroid cancer associated with neutrophilia and hypercalcemia were reviewed and discussed in this report.

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