# Trombocitopenia autoimune

> Página oficial: https://raras.org/doenca/trombocitopenia-autoimune
> Fonte: Raras.org — Banco de Dados de Doenças Raras em Português (CC BY-NC-SA 4.0)
> Última atualização: 2026-05-07

## Identificadores

- **ORPHA**: 71203 — https://www.orpha.net/en/disease/detail/71203
- **OMIM**: none — https://omim.org/entry/none

## Descrição clínica

Uma doença autoimune que leva à diminuição das plaquetas no sangue.

## Epidemiologia e herança


## Sinais e sintomas (32 fenótipos HPO)

- **Sangramento anormal** — HPO: HP:0001892
- **Anticorpo plaquetário positivo** — HPO: HP:0003454
- **Trombocitopenia** — HPO: HP:0001873
- **Icterícia** — HPO: HP:0000952
- **Epistaxe** — HPO: HP:0000421
- **Petéquias** — HPO: HP:0000967
- **Trombocitopenia autoimune** — HPO: HP:0001973
- **Suscetibilidade a hematomas** — HPO: HP:0000978
- **Palidez** — HPO: HP:0000980
- **Letargia** — HPO: HP:0001254
- **Fraqueza muscular** — HPO: HP:0001324
- **Dispneia** — HPO: HP:0002094
- **Fadiga** — HPO: HP:0012378
- **Síncope** — HPO: HP:0001279
- **Anemia hemolítica autoimune** — HPO: HP:0001890
- **Neutropenia na presença de anticorpos anti-neutrófilos** — HPO: HP:0001904
- **Trombose arterial** — HPO: HP:0004420
- **Positividade do anticorpo anti-ácido glutâmico descarboxilase** — HPO: HP:0025329
- **Positividade para anticorpo microssomal tipo 1 de fígado e rim** — HPO: HP:0030908
- **Sangramento vaginal anormal** — HPO: HP:0034263
- **Hemorragia cerebral** — HPO: HP:0001342
- **Positividade do anticorpo anti-insulina** — HPO: HP:0034062
- **Púrpura** — HPO: HP:0000979
- **Hemorragia gastrointestinal** — HPO: HP:0002239
- **Positividade do anticorpo anti-tireoperoxidase** — HPO: HP:0025379
- **Positividade do anticorpo anti-antígeno-2 de ilhotas** — HPO: HP:0034063
- **Positividade do anticorpo anti-antígeno plaquetário** — HPO: HP:4000170
- **Hematúria** — HPO: HP:0000790
- **Positividade do anticorpo anti-tireoglobulina** — HPO: HP:0032069
- **Sangramento gengival** — HPO: HP:0000225
- **Hemorragia ocular** — HPO: HP:0011885
- **Positividade do anticorpo anti-receptor de hormônio estimulador da tireoide** — HPO: HP:0034189

## Genes associados (1)

- **FCGR2C** — Low affinity immunoglobulin gamma Fc region receptor II-c [Candidate gene tested in]
  - Função: Receptor for the Fc region of complexed immunoglobulins gamma. Low affinity receptor. Involved in a variety of effector and regulatory functions such as phagocytosis of immune complexes and modulation

## Medicamentos em desenvolvimento (11)

- ROMIPLOSTIM — Fase Phase 4 (Thrombopoietin receptor agonist)
- DEXAMETHASONE SODIUM PHOSPHATE — Fase Phase 4 (Glucocorticoid receptor agonist)
- DEXAMETHASONE — Fase Phase 4 (Glucocorticoid receptor agonist)
- ELTROMBOPAG OLAMINE — Fase Phase 4 (Thrombopoietin receptor agonist)
- CORTISONE ACETATE — Fase Phase 4 (Glucocorticoid receptor agonist)
- FOSTAMATINIB DISODIUM — Fase Phase 4 (Tyrosine-protein kinase SYK inhibitor)
- PREDNISONE — Fase Phase 4 (Glucocorticoid receptor agonist)
- DECITABINE — Fase Phase 3 (DNA (cytosine-5)-methyltransferase 3A inhibitor)
- RITUXIMAB — Fase Phase 3 (B-lymphocyte antigen CD20 binding agent)
- PREDNISOLONE — Fase Phase 3 (Glucocorticoid receptor agonist)
- Fonte: https://platform.opentargets.org/disease/MONDO_0019098

## Ensaios clínicos ativos (9)

- **NCT07104565** [RECRUITING]: Study to Assess the Safety and Tolerability of Tafasitamab in Adult Participants With Primary Autoimmune Blood Cell Disorders — https://clinicaltrials.gov/study/NCT07104565
- **NCT07007962** [RECRUITING]: Study to Evaluate the Efficacy and Safety of Oral Rilzabrutinib in Adults With Immune Thrombocytopenia (ITP) Who Failed First-line Treatment — https://clinicaltrials.gov/study/NCT07007962
- **NCT07297563** [RECRUITING]: Anti-CD38 Monoclonal Antibody Versus Rituximab in the Management of Primary Immune Thrombocytopenia (ITP) — https://clinicaltrials.gov/study/NCT07297563
- **NCT07234019** [RECRUITING]: Rituximab Combining Anti-CD38 Monoclonal Antibody Versus Rituximab in the Management of Primary Immune Thrombocytopenia (ITP) — https://clinicaltrials.gov/study/NCT07234019
- **NCT04800458** [RECRUITING]: Contribution of Anti-platelet Antibodies Identified With MAIPA Assay in the Demonstration of the Auto-immune Character of a Thrombocytopenia at Diagnosis — https://clinicaltrials.gov/study/NCT04800458
- **NCT07362199** [RECRUITING]: Anti-CD38 Monoclonal Antibody Combined With Rituximab in the Management of Primary Immune Thrombocytopenia (ITP) — https://clinicaltrials.gov/study/NCT07362199
- **NCT07362238** [RECRUITING]: Daratumumab Versus Rituximab in the Management of Pediatric Primary Immune Thrombocytopenia (ITP) — https://clinicaltrials.gov/study/NCT07362238
- **NCT04562766** [ACTIVE_NOT_RECRUITING]: Study to Evaluate Rilzabrutinib in Adults and Adolescents With Persistent or Chronic Immune Thrombocytopenia (ITP) — https://clinicaltrials.gov/study/NCT04562766
- **NCT05023915** [ACTIVE_NOT_RECRUITING]: A Multicenter Randomized Open-label Study of Diammonium Glycyrrhizinate Enteric-coated Capsule Plus DXM Versus DXM in Treatment of ITP — https://clinicaltrials.gov/study/NCT05023915
- **NCT05369208** [COMPLETED]: Evaluation of Avatrombopag for the Treatment of Thrombocytopenia in Japanese Adults With Chronic ITP — https://clinicaltrials.gov/study/NCT05369208

## Doenças relacionadas (por similaridade fenotípica)

- [Microangiopatia trombótica](https://raras.org/doenca/microangiopatia-trombotica) — ORPHA:93573 — 26 sintomas em comum
- [Trombocitopenia imune](https://raras.org/doenca/trombocitopenia-imune) — ORPHA:3002 — 22 sintomas em comum
- [Doença mieloproliferativa crônica](https://raras.org/doenca/doenca-mieloproliferativa-cronica) — ORPHA:98274 — 15 sintomas em comum
- [Hemopatia mieloide](https://raras.org/doenca/hemopatia-mieloide) — ORPHA:171895 — 15 sintomas em comum
- [Anemia hemolítica autoimune](https://raras.org/doenca/anemia-hemolitica-autoimune) — ORPHA:98375 — 13 sintomas em comum
- [Síndrome Evans](https://raras.org/doenca/sindrome-evans) — ORPHA:1959 — 13 sintomas em comum
- [Leucemia mieloide aguda](https://raras.org/doenca/leucemia-mieloide-aguda) — ORPHA:519 — 12 sintomas em comum
- [Policitemia](https://raras.org/doenca/policitemia) — ORPHA:98427 — 10 sintomas em comum
- [Aplasia constitucional medular do adulto](https://raras.org/doenca/aplasia-constitucional-medular-do-adulto) — ORPHA:164823 — 10 sintomas em comum
- [Leucemia promielocítica aguda](https://raras.org/doenca/520) — ORPHA:520 — 10 sintomas em comum

## Importante

O Raras **não diagnostica e não prescreve**. Esta página é educativa e informativa.
Pacientes devem consultar profissionais de saúde qualificados para decisões clínicas.

---

**Citação sugerida**: Raras.org — Trombocitopenia autoimune. Disponível em: https://raras.org/doenca/trombocitopenia-autoimune
**Formato HTML**: https://raras.org/doenca/trombocitopenia-autoimune
**Formato RDF/Turtle**: https://raras.org/api/rdf?orpha=71203
