Resumo
Definição
História e exame físico
Principais fatores diagnósticos
- hemoglobinuria
- Budd-Chiari syndrome
- thrombosis
Outros fatores diagnósticos
- history of aplastic anemia
- fatigue
- anemia
- abdominal pain
- dysphagia and odynophagia
- erectile dysfunction
- dyspnea
- infections
- bleeding diathesis
- neurologic signs and symptoms
Fatores de risco
- aplastic anemia/hypoplastic bone marrow
- myelodysplasia
Investigações diagnósticas
Primeiras investigações a serem solicitadas
- urine dipstick
- urine microscopy
- CBC
- reticulocyte count
- serum LDH
- unconjugated bilirubin
- D-dimer
Investigações a serem consideradas
- haptoglobin
- flow cytometry for glycosylphosphatidylinositol anchor or anchored proteins
- fluorescent aerolysin
- immunotyping
- antiglobulin (Coombs) test
- serum iron, serum ferritin
- serum creatinine and BUN
Algoritmo de tratamento
classic PNH (hemolytic anemia): nonpregnant
classic PNH (hemolytic anemia): pregnant
PNH in the setting of another specific bone marrow disorder: nonpregnant
PNH in the setting of another specific bone marrow disorder: pregnant with thrombosis
PNH in the setting of another specific bone marrow disorder: pregnant without thrombosis
subclinical PNH: nonpregnant
subclinical PNH: pregnant
following acute clinical PNH with thrombosis: nonpregnant
Colaboradores
Autores
Ibrahim Ibrahim, MD
Associate Professor
Internal Medicine
UT Southwestern
Dallas
TX
Declarações
Taha Bat, MD
Assistant Professor
Internal Medicine
UT Southwestern
Dallas
TX
Declarações
TB serves on advisory boards for Alexion Pharmaceuticals. He is also the author of an article cited in this topic.
Agradecimentos
Dr Ibrahim and Dr Bat would like to gratefully acknowledge Dr Ilene Ceil Weitz and Dr Wendell F. Rosse, previous contributors to this topic.
利益声明
WFR serves on advisory boards and educational faculties for Alexion Pharmaceuticals. ICW receives speaker honoraria from and undertakes consultancy for Alexion Pharmaceuticals.
同行评议者
David Dingli, MD, PhD
Associate Professor of Medicine
Mayo Clinic
Rochester
MN
利益声明
DD declares that he has no competing interests.
Panayiotis D. Ziakas, MD, MSc, PhD
Department of Pathophysiology
Medical School
University of Athens
Athens
Greece
利益声明
PDZ declares that he has no competing interests.
鉴别诊断
- Autoimmune hemolytic anemia, warm antibody type
- Microangiopathic hemolytic anemia
- Paroxysmal cold hemoglobinuria
更多 鉴别诊断Guidelines
- Paroxysmal nocturnal hemoglobinuria
- Consensus guidelines to detect GPI‐deficient cells in paroxysmal nocturnal hemoglobinuria (PNH) and related disorders: Part 1 – clinical utility
更多 GuidelinesPatient information
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