Takashi Mizumoto

Autoimmune pancreatitis detected as a mass in the tail of the pancreas

A mass in the tail of the pancreas was detected in a 62‐year‐old male patient who had hypergammaglobulinaemia, and was positive for antinuclear antigen and anti‐SS‐A antibody. Endoscopic retrograde pancreatography revealed focal irregular narrowing of the main pancreatic duct in the tail of the pancreas. Dynamic computed tomography showed swelling of the pancreatic tail, which was enhanced on delayed phase. Autoimmune pancreatitis was suspected and corticosteroid therapy was commenced. This led to significant resolution of the pancreatic stricture. It is important to recognize this clinical entity as corticosteroid therapy may avoid unnecessary surgery.

CASE REPORT: Autoimmune Pancreatitis Detected as a Mass in the Head of the Pancreas with Contiguous Fibrosis Around the Superior Mesenteric Artery

Several cases of chronic pancreatitis associated with autoimmunity have been reported (1–3), and the concept of autoimmune pancreatitis was recently proposed (4, 5). This clinical entity is characterized by diffuse or focal swelling of the pancreas, irregular narrowing of the main pancreatic duct on endoscopic retrograde pancreatography (ERP), hypergammaglobulinemia, and the presence of autoantibodies, together with remarkable responsiveness to corticosteroid therapy. Some cases showing focal swelling or multiple focal masses that simulate pancreatic cancer or lymphoma have been reported by us and others (3, 6–10). Here, we report a case of autoimmune pancreatitis, detected as a mass in the head of the pancreas with a contiguous mass around the superior mesenteric artery, that was treated successfully with corticosteroid.

Chronic myeloid leukemia with minor‐bcr breakpoint developed hybrid type of blast crisis

Although a breakpoint in the minor breakpoint cluster region (m‐bcr) of the BCR gene is observed in about two‐thirds of patients with Philadelphia chromosome‐positive acute lymphoblastic leukemia, this type of genomic rearrangement occurs very rarely in chronic myeloid leukemia (CML). We describe here the eighth case of m‐bcr CML, and delineate unique clinical characteristics found in common to the 7 cases reported previously. Monocytosis with a low neutrophil/monocyte ratio resembling chronic myelomonocytic leukemia was the most striking feature of m‐bcr CML. Splenomegaly and basophilia were not conspicuous in chronic phase. A high percentage of immature granulocytes and low neutrophil alkaline phosphatase score were the findings in common with classical CML. Lymphoid and myeloid blast changes have been observed at and shortly after presentation so far. We found a hybrid type of blast crisis in the course of m‐bcr CML.Thus, m‐bcr CML may be a definite subtype of CML, exhibiting distinct clinical characteristics. The presence of fusion product of m‐bcr mRNA in an earlier myeloid cell may involve monocytic lineage in addition to myeloproliferative defects. Am. J. Hematol. 57:320–325, 1998.

Regulation of IgM anti-bovine γ-globulin antibody formation by helper and suppressor T cells in rabbits☆

Abstract Primary IgM anti-bovine γ-globulin (BGG) antibody formation was studied in rabbits subjected to 500 or 900 R of whole body X-irradiation in combination with one or more of the following conditions: appendectomy (Ax), thymectomy (Tx), shielding of appendix (As) or bone marrow (Bs) during irradiation (X), transfer of 10 9 appendix or thymus cells (Ac or Tc), and immediate immunization with 200 μg of alum-precipitated BGG iv. The IgM antibody response was well preserved in AsX rabbits in comparison with X 500 rabbits. Conversely, the response was suppressed in AxX 500 rabbits although transfer of autologous appendix cells to these animals (AxX 500 .Ac) restored the response. In the group receiving 900 R, there was variable restoration in individual AxX.Ac and AxBsX.Ac rabbits and little or none in AxTxX.Tc, AxTxBsX.Tc, and AxTxBsX.Ac animals. However, simultaneous injections of autologous thymus cells and appendix lymphocytes (AxTxX.Ac.Tc) restored the response completely. The IgM response was somewhat enhanced in AsTxX animals and returned again to the level of normal controls by injecting these animals with autologous thymus cells. However, marked suppression was seen when AsTxX rabbits were injected with neonatal allogeneic thymus cells. A remarkable enhancement of IgM antibody formation was induced in normal rabbits by injection of PHA into the appendiceal artery. On the basis of these results and of histological observations, it was suggested that the rabbit appendix represents a large pool of both B and T cells and that appendix-derived B cells participating in IgM anti-BGG antibody formation are under the regulation of both helper and suppressor T cells.

B and T Cells in the Lymphoid Tissues of Human Appendix

Human appendix lymphoid cells (HAL) react very strongly to stimulation with concanavalin A, strongly to stimulation with phytohaemagglutinin, and weakly but definitely to stimulation with lipopolysaccharide. From the results of rosette formation assay, cytotoxicity tests with anti-T cell antiserum or anti-B cell antiserum, cell surface or intracellular immunoglobulin staining with fluorescein-conjugated rabbit anti-Fab of human immunoglobulin serum, and plaque-forming cell (PFC) assay, it was concluded that human appendix lymphoid tissue is a B cell pool but includes T cells. However, both direct and indirect PFC could not be significantly demonstrated against sheep red blood cells in a 5-day HAL culture.

Squamous Cell Carcinoma Associated with Disturbance of the IgA System

A case of cell carcinoma of the skin associated with disturbance of the IgA system is described. Immunological observations revealed a marked low level of serum IgA and disturbance of the secretory IgA synthesis in the rectal mucosa. After the surgical removal of the skin tumor, the serum level of IgA was increased to half the normal-range level. The relationship of the disturbance of the IgA system to the occurrence of squamous cell carcinoma is discussed.

[An autopsied case of G-CSF producing lung cancer with bullous pemphigoid and hyper gammaglobulinemia].

症例は78歳男性,全身に多数の水疱,びらんを認め水疱性類天疱瘡(bullous pemphigoid: BP)と診断された.血清IgG 3,870mg/dl, γG 45.6%と高値であった.剖検で左肺に未分化型大細胞癌を認め,血中G-CSFは2,680pg/mlと著明に高値を示した.肺癌はG-CSF染色で陽性であり, G-CSF産生肺癌と診断した.間接蛍光抗体法では皮膚基底膜にIgGの沈着を認め,さらに肺癌はIgG染色で陽性であった. BPと肺癌の関連性を考えるうえで興味ある症例と考え報告した.

A case of Gaucher's disease complicated with liver failure.

本邦では遭遇する機会の少ないロシア系ユダヤ人の成人型Gaucher病を経験した.症例は46才男性. 6才時脾摘.全身倦怠感,黄疸にて入院.検査で肝障害著明.アンモニア値508μg/dlと著増.酸フォスファターゼ42.7Uと高値, IgM-HA抗体陽性.意識障害進行し死亡.剖検時の骨髄スタンプ標本でGaucher細胞を多数認めた.肝4700gと腫大し, Gaucher細胞が高度に増生していた.剖検肝の分析では,全脂質の増加あり,薄層クロマトグラフィーでglucocerebrosideが全脂質の87%と著増していた.また人工基質で測定したβ-glucosidase活性は15.4%と低下していた.したがって,本症例は, Gaucher病による肝障害にA型肝炎が合併し,肝不全にて死亡したと考えられた.

Surface antigens of murine IgM and IgG forming cells.

Two kinds of cytotoxic antibodies have been obtained by Immunizing rabbits with the cultured lines of either IgM‐forming tumor cells (MOPC‐104E) or IgG‐forming ones (MOPC‐31B). Antl‐MOPC‐104E antiserum and anti‐MOPC‐31B antiserum reacted specifically with direct and indirect hemolytic plaque forming cells, respectively. The specificities became manifest after absorption with liver powder, peripheral blood cells and the tumor cells producing a different class of immunoglobulin. The indirect plaque forming cells acquired the specific surface antigens in the stage of antibody secretion or shortly before It.