Hiroo Furukawa

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.

The physicochemical properties and the process of pyrogel formation of IgD pyroglobulin.

The extremely rare IgD pyroglobulin caused opalescence, lost its antigenicity and acquired more negative charge when heated for 30 min at 56 degrees C. Exposure to 60 degrees C for 60 min produced a firm gel. The thermoprecipitability was abolished by the treatment with guanidine, urea and sodium dodecyl sulfate, but not by 2-mercaptoethanol or neuraminidase, and required the presence of both heavy and light chains. However, amino acid analysis showed abnormalities only in the heavy chain. We hypothesize that the irreversible heat-induced aggregation of IgD pyroglobulin is the result of a conformational change, triggered by light chain, which increases the molecular hydrophobicity.

THREE-DIMENSIONAL RECONSTRUCTION OF THE MUCOSA FROM SEQUENTIAL SECTIONS OF BIOPSY SPECIMENS FOR UNDERSTANDING DISEASE PROGRESS IN ULCERATIVE COLITIS

Background: This paper describes the creation of 3‐D computer graphic images of the mucosal crypt of ulcerative colitis (UC) and the resulting observations, which may have great significance to our understanding of the mechanisms involved in the progress of this disease.

FAMILY STUDY SHOWING A CLOSE ASSOCIATION OF HLA DPB1*0202/0901 WITH ULCERATIVE COLITIS

Genetic disposition and environmental factors are closely related to the occurrence of ulcerative colitis (UC). The HLA haplotypes of major histocompatibility complex (MHC) Class II are considered to be the most important genetic factors in UC. The current report focuses on the genetic human leukocyte antigen (HLA) of a family in which UC crises have occurred in five of six members with identical MHC Class II haplotypes. Haplotypes A24/26 (A24/‐), BW4/6 (BW4/‐) and DQ1/3 (DQ1/‐) were found in all six members of the family. In DNA typing, characteristic loci common to this family were seen in DRB11502 and DQB10601. From the genetic investigation of this family, we concluded that HLA haplotypes are the most important genetic factors in the occurrence of UC.

Three-dimensional reconstruction of the mucosa from sequential sections of biopsy specimens of patients with ulcerative colitis: Relationship between crypt structure and vascular architecture

Background:  In a previous paper, the stereographic reconstruction of the crypt structure of ulcerative colitis using the RATOCK System was described. The relationship between the blood vessels and the crypt structure is the focus of the current paper, using two kinds of tissue staining color in which the color differs. Stereographic images make the relationship between the crypt structure and blood vessel distribution understandable at a glance.

[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.

Studies on colonic mucus release.I . Normal colon.

大腸粘膜における局所の免疫反応時のmucus release (粘液放出)について検討した.小腸を場とした抗原抗体反応では,経十二指腸的に動物に抗原を投与し,同一抗原でchallengeしたときに, mucus releaseが正常小腸で増加するというLake A, M.,らの報告がある.これに対して著者らは,ラットの正常大腸粘膜に経直腸的にBSA (bovine serum albumine)による免疫を繰り返した後,免疫対応または非対応抗原で経直腸的challengeを行い,抗原抗体反応によるmucus releaseについて検討した.その結果,小腸と異なり正常大腸では局所免疫で, mucus releaseの増加は対照と差がみられず,正常粘膜では小腸と大腸の局所的抗原抗体反応に差異があることが判明した.

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.