Familial relapsing thrombotic micro-angiopathy in two siblings

Anna Hazani, A Hazani, A Eldor, Amiram Eldor, Y Shechter, Yael Shechter, A Etzioni, Amos Etzioni
European journal of pediatrics · 1996 · vol. 155(2) , pp. 99–101 · doi:10.1007/bf02075759 · PMID:8775222 · W2083429992
Case report OA: closed CC0
View on OpenAlex View on PubMed View at publisher
⚙ AI-generated summary by qwen3.7-flash, 2026-10-11 ⓘ

This report describes two siblings with familial relapsing thrombotic microangiopathy, noting that danazol reduced disease severity in the girl while plasma exchange improved the boy's condition.

One-sentence paraphrase of the abstract; not a substitute for reading it. No clinical advice. How this works

Abstract

UNLABELLED: Two siblings, a 12-year-old girl and a 7-year-old boy, had recurrent episodes of haemolytic-uraemic syndrome/thrombotic thrombocytopenic purpura, manifested mainly by thrombocytopenia and micro-angiopathic haemolytic anaemia. During 11 years of follow up the girl responded only to steroids, whereas many other therapeutic modalities were ineffective. Following treatment with low dose danazol relapses became fewer and of diminished severity and completely subsided after 6 months. The boy started his illness with signs of haemolytic uraemic syndrome and later developed neurological manifestations. During a 6 year follow up he responded only to plasma exchange. Although chronic thrombocytopenia persisted during the past 3 years, the boy's clinical condition improved. CONCLUSION: A family with two children with recurrent episodes of thrombotic thrombocytopenic purpura is described. In one child danazol could have had a beneficial effect.

My notes (saved in your browser only)

MeSH descriptors

Anemia, Hemolytic Thrombocytopenia Anemia, Hemolytic Anemia, Hemolytic Anemia, Hemolytic Anemia, Hemolytic Child Danazol Danazol Estrogen Antagonists Estrogen Antagonists Female Humans Male Plasma Exchange Recurrence Thrombocytopenia Thrombocytopenia Thrombocytopenia Thrombocytopenia

Citation neighborhood (no data yet)

We don't have any in-corpus citations linked to this paper yet. The paper's references may be in our DB but unresolved to ``paper_id`` (resolution happens at ingest when the cited DOI matches a row we already have). Run the cross-source citation reconcile pass to retry.

References (16)

Source provenance

openalex
last seen: 2026-05-11T05:51:57.177246+00:00
pubmed
last seen: 2026-10-08T21:59:55.756364+00:00
License: CC0 · commercial use OK