A series of comprehensive survey study was conducted during 1974∼1976 under the sponsorship of Hemolytic Anemias Research Committee, the Ministry of Health and Welfare, Japan (Chairman, Professor Shiro Miwa, Yamaguchi University), with the principal purposes of evaluating the current status of the two representative types of hemolytic anemia, i. e. autoimmune hemolytic anemia (AIHA) and hereditary spherocytosis (HS).
Relevant clinical and laboratory data were generously provided for analysis by 160 major institutions and clinics on 141 cases with AIHA and 144 cases with HS. Extensive analysis of these materials yielded sufficiently detailed informations on a variety of clinical aspects of the disease, which are of essential importance for the adequate understanding and effective management of these diseases.
Included were onset-age distribution, heredity and familial occurrence, clinical picture at diagnosis, routine and special hematological examinations, biochemical and serological features, treatment and its effectiveness, side effects, clinical course, and overall prognosis.
Characteristic features were outlined individually for AIHA and HS, while several problems awaiting forthcoming clarification were pointed out, including the particular need for elucidation of the eventual development and long-term prognosis of AIHA and also for the recognition and management of HS during neonatal period.

抄録全体を表示

Fifty-seven adult patients with relapsing acute non-lymphocytic leukemia (ANLL) who underwent reinduction chemotherapy during 1971∼1983 were analysed with special referance to comparative effectiveness of two treatment shedules.
Twenty-seven patients received reinduction chemotherapy after the first relapse with regimen A in which administration of DCMP, ACMP or BH-AC·DMP was continued until the reduction of leukemic cells to a certain target level was achieved. Twenty-five patients were evaluable (Group A). The remaining thirty patients were treated with regimen B (repeated 4 consecutive day therapy of DCMP or AVCP) after their first relapse. Twenty-six patients were evaluable (Group B).
Complete remission (CR) rate was 16/25 (64%) in Group A, whereas it was 3/26 (12%) in Group B. Thus CR rate in Group A was significantly superior to that in Group B (p<0.01). The CR rate of Group A patients was close to the figure attained by the previousely untreated patients (73%). Fifty percent remission duration and survival time from diagnosis in Group A were 363 and 560 days, while those were 90 and 169 days in Group B, respectively. Significant difference (p<0.01) was observed in median duration of survival between the two groups.
These observations indicate that regimen A is as effective for the reinduction of relapsing ANLL as for the first remission induction.

抄録全体を表示

The Clinicopathological features of 51 patients with untreated megaloblastic anemia due to vitamin B₁₂ deficiency were analysed. The material consisted of 40 patients with Addisonian pernicious anemia (PA), 10 with postgastrectomy B₁₂ deficiency (PG) and one with blind-loop syndrome. No sex preponderance was noted in PA patients with mean age of 63 years. Mean Broca's indices for PA and PG patients were 88.9% and 77.2%, respectively, showing a poorer nutritional status in PG patients. The mode of onset, clinical symptoms, hematological findings including bone marrow morphology, blood chemistry, ferro-erythrokinetic data and nervous system involvement were all essentially analogous in PA and PG patients. The spleen was palpably enlarged in 25% of PA patients at diagnosis. The correlations between various laboratory parameters were examined.

抄録全体を表示

The cooperative study group on “Preleukemia” supported by Grant in-Aid for Cancer Research from the Ministry of Health and Welfare, Japan, designated “refractory anemia with excess of blasts” as one of the project studies in 1980, with the views of clarifing whether patients with RAEB exist in Japan as well as Europe and whether they, if exist, have any particular clinical findings, and of establishing a scoring method for distinguishing RAEB from other hematological diseases.
Clinical records of suspected RAEB, which had been conceived by Dreyfus, were collected by every member of the study group. This investigation proved the existence of RAEB patients in Japan. Clinical and laboratory findings of fifty eight cases were analysed for the scoring method. The following 13 items were picked up for the criteria and were respectively given scores according to their significance;
as bone marrow findings
1) nucleated cell count more than 50×10⁹/L score 12
2) 7 to 30% of blasts plus promyelocytes score 12
3) steady state of bone marrow findings more than 6 months score 12
4) 10 to 50% of erythroblasts score 8
5) more than 15% of myelocytes+metamyelocytes+bands+segmented neutrophils score 8
6) less than 20% of lymphocytes score 8
7) morphological abnormalities more than two series in bone marrow elements score 8
as peripheral blood findings
8) more than 50×10⁹/L of platelets score 8
9) more than 0.5×10⁹/L of neutrophil counts score 4
10) less than 5% of immature granulocytes score 4
11) less than 60% of lymphocytes score 4
12) more than 180 of neutrophil AP score score 4
13) age older than 50 score 8
The patients with scores 90 to 100 in total were regarded as definite RAEB, scores 80 to 89 probable RAEB, scores 70 to 79 possible RAEB, and scores less than 69 impossible. The scoring method was applied to other hematological disorders and revealed that the patients with leukemia of M1, M2, M3, M4, or M5 types according to FAB classification and those with aplastic anemia had scores less than 69 without any exception. However, scores of some cases with M6 and primary acquired sideroblastic anemia ranged from 70 to 76.
From these results, the scoring method is presented as to be instrumental in making the diagnosis of RAEB as a supportive means.

抄録全体を表示

急性白血病という疾患自体が宿主側からみれば免疫不全状態である上に, 末梢成熟顆粒球数の減少も伴い感染症を合併しやすいことは周知である. また, 急性白血病の治療は現在‘Total cell ki11’の治療理念に基づき強力な多剤併用療法がおこなわれており, このような医原的因子も加わり本症の治療過程で感染症は必発するといつても過言ではない. したがつて, 合併感染症の治療の成否は急性白血病の予後を左右する要因の1つである.
近年, 急性白血病とくに小児急性リンパ性白血病の治療成績は著しく向上し, 約30%は根治しうるといわれている. 一方, 成人急性非リンパ性白血病の5年生存率は未だ低率とはいえ, 長期生存例は増加の一途をたどつている. このような治療成績の向上は各種抗白血病剤の開発と, その投与方法の改善に負うところが大きいが, 治療過程に合併する重篤な感染症や出血に対する支持療法の進歩も忘れることはできない. とはいえ, 化学療法剤の普及に伴い薬剤耐性菌が出現し, その蔓延の速度は極めて早いことが指摘されている. 一方, それに対抗しうる新しい化学療法剤の開発に努力が重ねられているのが現状である. 幸い, 従来のセファロスポリンでは抗菌力が期待できなかつたEnterobacter, citrobacter, Indole陽性ProteusやHaemophilus influenzaeにもすぐれた抗菌力を示すCefotiam (CTM, Pansporin®) が開発され市販されるに至つた. 著者らは急性白血病合併感染症に対してCTMを治験する機会を得たのでその成績を報告する.

抄録全体を表示

60歳時特発性血小板減少性紫斑病と診断され, それ以来 Prednisone 10mg/日の投与を受けている72歳の男性. 62歳頃より次第に両側下肺野にびまん性の網状陰影が認められるようになった. 慢性型の間質性肺炎と診断されたが原因は不明. 下肺野背側の蜂窩肺の進行とともに, 上肺野前胸部側の嚢胞性変化も目立つようになってきた. 特発性血小板減少性紫斑病に合併し慢性に経過した間質性肺炎報告例は少なく, また嚢胞性変化が下肺野では背側に上肺野では前胸部側に生じた点が, 意義深く思われた.

抄録全体を表示

小児科診療において, 上気道感染は最もしばしば遭遇するものであり, 中でも膿栓または偽膜を伴なう急性扁桃・咽頭炎は, 細菌感染との関連が高率である疾患の1つである。
今回, 抗生剤セファレキシンの持続性製剤(S-6435およびS-6436)をシオノギ製薬K. K.から提供を受け, 臨床的検討の機会を得たので, 扁桃・咽頭炎の患者を選び, 本剤の臨床効果および検出菌種とその感受性について検索をおこなつたので, その成績を報告する。

抄録全体を表示

5種類のアルカリ金属チオシアン酸塩の融解状態におけるモル容積を石英ガラス製の膨張計を用いて測定し,温度の関数としての実験式で表わした。また,これらの融解浴の屈折率を石英ガラス製のプリズムを用いて,ゴニオメーター法で測定し,波長と温度の関数としてCauchyの実験式として表わした。

抄録全体を表示

症例は32才の日本人男性で, 17才頃より貧血を指摘されていたが,全身倦怠感を主訴に近医を受診し,貧血と脾腫を指摘され精査目的で当科へ入院した.入院時,尿検査においてウロビリノーゲンの陽性所見を認めるもヘモジデリン等の異常は認めず,末梢血でRBC 463万/mm³, Hb 9.lg/dl, Ht 28%, MCV 60μ³, MCH 20rr,血液像では赤血球大小不同,奇型,標的細胞が認められた.血液生化学検査において,軽度の間接型ビリルビンの上昇を認める以外には異常を認めず,また血清鉄88μg/dl,総鉄結合能225μg/dlであつた.以上より溶血性貧血を疑いさらに検査をすすめた. Heinz小体生成試験が陽性を示した以外,熱変性試験, sugar-water試験,直接,間接クームス試験はいずれも陰性であつた. hemoglobin電気泳動の結果〔A₂ 6.2%, F21.5%, abnormal hemoglobin (-)〕よりβ-thalassemiaを疑い, ¹⁴C-leucineによるβ鎖/α鎖の合成比を求めたところ, 0.42とβ鎖合成の抑制が認められた.以上,家族歴,臨床症状,これら検査成績よりβ-thalassemia intermediaと診断した.本邦におけるthalassemiaは現在までに47家系の報告がみられるが, β-thalassemia intermediaとして報告されたのは本症例を含め5例のみであり,非常にまれな症例と考えられる.

抄録全体を表示

In a study on proliferation kinetics of erythroblasts in myeloid leukemia, the following results were obtained :
1) In the untreated, acute type, the activity of erythropoietin responsive cells (ERC) in the bone marrow was markedly decreased. There was a significant, positive correlation between ERC activity and relative erythroblast counts in the bone marrow. In these cases, the labeling index (LI) with ³H-thymidine of the total erythroblasts was significantly decreased. The decreased in LI was more striking in the cases where the erythroblasts resembling megaloblasts (PAS negative) were found in the bone marrow smears. LI in the basophilic erythroblasts remained within normal range, while the decrease in LI was conspicuous and statistically significant in polychromatic erythroblasts. In remission, ERC activity was raised to normal or above normal. LI was also normalized. The DNA synthesis time of erythroblasts in the untreated acute type was 12.9 + 0.4 hours, which did not differ from that of normal erythroblasts. The decreased LI was, therefore, considered to indicate that the polychromatic erythroblasts had a long G₁ phase or that those which got into the G₀ phase increased. In these cases, it was revealed that increase in number of erythroblasts with nuclei of small diameter resulted in a decreased labeling index.
2) In chronic myeloid leukemia, ERC activity and LI of erythroblasts showed no particular changes.
3) There occurred no striking changes in the LI of erythroblasts of cancer patients.
4) From the results presented above, it was concluded that there were not only quantitative but also qualitative changes in the erythropoiesis in acute myeloid leukemia.

抄録全体を表示

The present series of experimental studies was undertaken to give solutions to the following questions: (1) can antibiotics administered intravesically be abiorbed from the wall of the bladder?,(2) if so, does their intravesical injection prove to be effective in the treatment of cystitis? and (3) if this true, can their intravesical injection provide an advantage over their systemic use since a larger dose can be given at a higher concentration by the former method of administration?
If an antibiotic is transferred in a substantial amount to the blood following its intravesical administration, the end result of such medication may be identical with that of administration by systemic routes since the antibiotic transferred into the circulation will eventually be recovered in the urine after being excreted by the kidney or through the wall of the bladder. It may be said, then, that a large intravenous or intramuscular: dose can be used more advantageously than cumbersome, frequent intravesical doses, although. this may not always be true with all types of antibiotics. If, on the other hand, an intravesically administered antibiotic is not transferred to the blood in substantial quantities or is promptly eliminated from the circulation after being transferred to it and, moreover, proves to be comparable to its systemic administration in terms of therapeutic efficacy against infection, this particular mode of antibiotic therapy may be suited for the treatment of patients with hepatic and/or renal impairment, those in whom injection is impractical as well as piegnant women
These considerations led us to conduct basic and clinical studies of sulbenicillin (SBPC) in order to determine whether this antibiotic can permeate into the blood or can pass through bladder walls following intravesical injection, to what extent it is recovered in urine and whether or not it is clinically effective

抄録全体を表示

Candidiasis of the liver developed during the initial remission induction treatment in two patients with acute promyelocytic leukemia. Both patients experienced abdominal pain, high fever and hepatomegaly, and the second patient developed strikingly elevated serum alkaline phosphatase (ALP). Ultrasonography demonstrated intrahepatic lesions of increased echogenecity and CT scan revealed low-density areas without contrast enhancement. A quantitative measurement of anticandida antibody level confirmed that deep-seated candida infection had occured. The second patient underwent treatment with amphotericin B, 5-FC and minocycline without success. It is important to make prophylactic use of amphotericin B in patients with acute leukemia even during initial remission induction therapy.

抄録全体を表示