Paroxysmal nocturnal hemoglobinuria

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Paroxysmal nocturnal hemoglobinuria

MRCP revision

MRCP revision

Acute intermittent porphyria
Autoimmune hemolytic anemia
Glucose-6-phosphate dehydrogenase (G6PD) deficiency
Sickle cell disease (NORD)
Alpha-thalassemia
Beta-thalassemia
Anemia of chronic disease
Iron deficiency anemia
Sideroblastic anemia
Aplastic anemia
Immune thrombocytopenia
Polycythemia vera (NORD)
Antiphospholipid syndrome
Antithrombin III deficiency
Factor V Leiden
Protein C deficiency
Protein S deficiency
Hemophilia
Vitamin K deficiency
Disseminated intravascular coagulation
Heparin-induced thrombocytopenia
Von Willebrand disease
Hemolytic-uremic syndrome
Thrombotic thrombocytopenic purpura
Paroxysmal nocturnal hemoglobinuria
Myelodysplastic syndromes
Myelofibrosis (NORD)
Acute leukemia
Chronic leukemia
Hodgkin lymphoma
Non-Hodgkin lymphoma
Neuroblastoma
5-alpha-reductase deficiency
Androgen insensitivity syndrome
Congenital adrenal hyperplasia
Kallmann syndrome
Thyroglossal duct cyst
Pancreatic neuroendocrine neoplasms
Zollinger-Ellison syndrome
Multiple endocrine neoplasia
Carcinoid syndrome
Hyperparathyroidism
Hypoparathyroidism
Hyperprolactinemia
Pituitary adenoma
Prolactinoma
Delayed puberty
Premature ovarian failure
Constitutional growth delay
Growth hormone deficiency
Hypopituitarism
Hypoprolactinemia
Pituitary apoplexy
Sheehan syndrome
Diabetes insipidus
Syndrome of inappropriate antidiuretic hormone secretion (SIADH)
Thyroid eye disease (NORD)
Thyroid storm
Toxic multinodular goiter
Euthyroid sick syndrome
Postpartum thyroiditis
Riedel thyroiditis
Subacute granulomatous thyroiditis
Thyroid cancer
Bacillus cereus (Food poisoning)
Campylobacter jejuni
Clostridium difficile (Pseudomembranous colitis)
Escherichia coli
Helicobacter pylori
Listeria monocytogenes
Salmonella (non-typhoidal)
Salmonella typhi (typhoid fever)
Shigella
Staphylococcus aureus
Vibrio cholerae (Cholera)
Yersinia enterocolitica
Coxsackievirus
Cytomegalovirus
Herpes simplex virus
Mumps virus
Ascending cholangitis
Gallstone ileus
Alcohol-associated liver disease
Alpha 1-antitrypsin deficiency
Budd-Chiari syndrome
Cholestatic liver disease
Cirrhosis
Gilbert's syndrome
Hemochromatosis
Hepatic encephalopathy
Viral hepatitis
Jaundice
Non-alcoholic fatty liver disease
Portal hypertension
Primary biliary cholangitis
Primary sclerosing cholangitis
Reye syndrome
Wilson disease
Acute pancreatitis
Chronic pancreatitis
Pancreatic pseudocyst
Hirschsprung disease
Intussusception
Ischemic colitis
Microscopic colitis
Necrotizing enterocolitis
Protein losing enteropathy
Short bowel syndrome (NORD)
Small bowel bacterial overgrowth syndrome
Small bowel ischemia and infarction
Tropical sprue
Volvulus
Whipple's disease
Autoimmune hepatitis
Celiac disease
Eosinophilic esophagitis (NORD)
Benign liver tumors
Hepatocellular adenoma
Hepatocellular carcinoma
Familial adenomatous polyposis
Juvenile polyposis syndrome
Peutz-Jeghers syndrome
Gastric cancer
Abdominal hernias
Achondroplasia
Developmental dysplasia of the hip
Muscular dystrophy
Osteomalacia and rickets
Osteoporosis
Paget disease of bone
Gout
Osteoarthritis
Compartment syndrome
Ankylosing spondylitis
Dermatomyositis
Juvenile idiopathic arthritis
Limited systemic sclerosis (CREST syndrome)
Mixed connective tissue disease
Polymyositis
Psoriatic arthritis
Raynaud phenomenon
Reactive arthritis
Rheumatoid arthritis
Sjogren syndrome
Systemic lupus erythematosus
Septic arthritis
Spondylitis
Baker cyst
Bursitis
Polymyalgia rheumatica
Temporomandibular joint dysfunction
Transient synovitis
Bone tumors
Fibromyalgia
Sciatica
Carpal tunnel syndrome
Rotator cuff tear
Prions (Spongiform encephalopathy)
Adenovirus
Eastern and Western equine encephalitis virus
Epstein-Barr virus (Infectious mononucleosis)
HIV (AIDS)
JC virus (Progressive multifocal leukoencephalopathy)
Lymphocytic choriomeningitis virus
Measles virus
Poliovirus
Rabies virus
Varicella zoster virus
West Nile virus
Cerebral palsy
Neurofibromatosis
Tuberous sclerosis
Erb-Duchenne palsy
Creutzfeldt-Jakob disease
Neurogenic bladder
Treponema pallidum (Syphilis)
Vitamin B12 deficiency
Acoustic neuroma (schwannoma)
Glaucoma
Amyloidosis
Focal segmental glomerulosclerosis (NORD)
Membranous nephropathy
Minimal change disease
Renal tubular acidosis
Nephroblastoma (Wilms tumor)
Non-urothelial bladder cancers
Transitional cell carcinoma
Renal cell carcinoma
Urinary incontinence
Renal cortical necrosis
Nephrotic syndromes: Pathology review
Renal tubular defects: Pathology review
Renal tubular acidosis: Pathology review
Cardiac preload
Bacillus anthracis (Anthrax)
Bacteroides fragilis
Bordetella pertussis (Whooping cough)
Corynebacterium diphtheriae (Diphtheria)
Coxiella burnetii (Q fever)
Francisella tularensis (Tularemia)
Moraxella catarrhalis
Bacterial epiglottitis
Laryngomalacia
Bronchodilators: Beta 2-agonists and muscarinic antagonists
Bronchodilators: Leukotriene antagonists and methylxanthines
Cytomegalovirus infection after transplant (NORD)
Graft-versus-host disease
Post-transplant lymphoproliferative disorders (NORD)
Transplant rejection
Anaphylaxis
Food allergy
Type I hypersensitivity
Hemolytic disease of the newborn
Pemphigus vulgaris
Type II hypersensitivity
Serum sickness
Type III hypersensitivity
Contact dermatitis
Type IV hypersensitivity
Atopic dermatitis
Lichen planus
Psoriasis
Erythema multiforme
Stevens-Johnson syndrome
Bullous pemphigoid
Acne vulgaris
Skin cancer

Key Takeaways

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired, life-threatening condition characterized by the destruction of red blood cells by the complement portion of the immune system.

This destructive process is a result of a defect in the formation of surface proteins on the red blood cell, which normally functions to inhibit such immune reactions. Common symptoms of PNH are fatigue due to anemia, abdominal pain, and thrombosis, which is the major cause of death. There can also be jaundice from the accumulation of bilirubin, and characteristic dark-colored urine.

The management of PNH involves drugs like eculizumab, a monoclonal antibody that blocks the activity of the complement system, anticoagulation therapy in case of thrombosis, blood transfusion, and supportive management as needed.