定型的な血栓性栓球減少性紫斑病の1剖検例

A TYPICAL CASE OF THROMBOTIC THROMBOCYTOPENIC PURPURA (T. T. P.)

I．はじめに

　血栓性栓球減少性紫斑病（Thrombotic thrombo—cytopenic purpura以下T．　T．　P．）は1925年Mosch—cowitzs^13）によつて初めて報告され，臨床的には発熱，皮下出血，溶血性黄疽，栓球減少および一過性の精神神経症状を呈する疾患であり，その経過はきわめて急激で，数日ないしは数週問で死亡することが多い。病理解剖では肉眼的にたいした病変はみられないが，組織学的には脳を含めた全身臓器の細小動脈に多発性の血栓形成があり，その形態や部位がきわめて特異的であるため，定型例では診断は容易である。すでに本症の報告は欧米では170例にも達しているといわれるが^11）本邦では猪瀬^9）が1949年に「脳症状を呈した紫斑病の1特異例」として発表したのが最初で，今日までに13例の剖検例（うち生前にT．　T．　P．と診断，あるいはT．　T．　P．が疑われたものは2例^10）16））が報告されているにすぎない。最近Adel—sonら^1），March^12），河野ら^11）によつて詳細な総説がなされており，本症に関する臨床的ならびに病理学的関心が高まつてきたが，同時に他疾患との合併症例，またはまぎらわしい症例の報告も増えてきている。以上の意味から，最近われわれが経験した定型例をここに提示し，少しく検討を加えてみたい。

On Sept. 6, 1962, a 52 year old office-man was admitted to the hospital with the chief complaints of transiently repeated unconsciousness and motor disturbances. His parent and 3 among 9 sibs had died of apoplexia. At the age of 29 years he had had blast injury, being partially paralysed on the right upper limb. Four years prior to admission he had undergone segmentary pulmonectomy (S₁₊₂, S₃) on the left and the next year exstirpasion of right upper lobe, owing to pulmonary tuberculosis. Three weeks before he first noticed general tiredness, nausea and numbness of his right hand. Two days before he had a transient attack of right hemiplegia and dysphasia. On admission he was delirious and icteric and multiple ecchymoses were present on the axillae, chest wall and upper limbs. A probable diagnosis of T. T. P. was made, owing to transient neuropsychiatric symptoms, hemorrhagic diathesis and blood findings (anemia 231 millions, thrombocytope-nia 60,000, prolonged bleeding time and normal coagulation time). Gradually the condition becoming worse, he expired under status epilepticus on 7th hospital day.

The postmortem histological examination disclosed multiple hyaline thrombus formation in the arterioles of various organs (Of marked degree in the brain, spleen, heart, kidneys, adrenals, pancreas, neurohypo-physis etc. Of moderate degree in the stomach intestine, testes, bone marrow etc. Of slight degree of absent in the lungs, liver, prossate tongue, sali-vary glands, lympnodes etc.). The thrombus was adherent to the vessel wall on one side, accompanied by subinitimal fibrinoid degeneration and on the opposit side the wall became enlarged by aneurysma formation. Megacaryocytes were abundant in the bone marrow and also in the lung capillaries.

The clinical symptoms have been explained from pathological findings and the pathogehesis of T. T. P. has been discussed, being compared with collagen diseases.