Paroxysmal nocturnal hemoglobinuria

Jump to: navigation, search

For patient information click here

Paroxysmal nocturnal hemoglobinuria
ICD-9 283.2
OMIM 311770
DiseasesDB 9688

Paroxysmal nocturnal hemoglobinuria Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Paroxysmal nocturnal hemoglobinuria from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Laboratory Findings

X Ray

CT

MRI

Echocardiography or Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Paroxysmal nocturnal hemoglobinuria On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Paroxysmal nocturnal hemoglobinuria

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Paroxysmal nocturnal hemoglobinuria

CDC on Paroxysmal nocturnal hemoglobinuria

Paroxysmal nocturnal hemoglobinuria in the news

Blogs on Paroxysmal nocturnal hemoglobinuria</small>

Directions to Hospitals Treating Paroxysmal nocturnal hemoglobinuria

Risk calculators and risk factors for Paroxysmal nocturnal hemoglobinuria

Editor(s)-in-Chief: C. Michael Gibson, M.S.,M.D. [1] Phone:617-632-7753; Robert Killeen, M.D. [2]

Synonyms and keywords: PNH; Marchiafava-Micheli disease; paroxysmal nocturnal haematuria

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Paroxysmal nocturnal hemoglobinuria from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

History and Symptoms | Physical Examination |Laboratory Findings | X Ray | CT | MRI | Echocardiography or Ultrasound | Other Imaging Findings | Other Diagnostic Studies

Treatment

Medical Therapy | Surgery | Primary Prevention | Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case #1

v • d • e
Pathology: hematology, myeloid hematologic disease (primarily D50-D77, 280-289)
RBCs/
hemoglobinopathy
+ Polycythemia - Macrocytosis
-
Anemia
Nutritional Iron deficiency anemia (Plummer-Vinson syndrome), Megaloblastic anemia (Pernicious anemia)
Hemolytic
Hereditary enzyme: G6PD Deficiency - Pyruvate kinase deficiency - Triosephosphate isomerase deficiency

hemoglobin: Thalassemia - Sickle-cell disease/trait

membrane: Hereditary spherocytosis - Hereditary elliptocytosis - Hereditary stomatocytosis
Acquired Autoimmune (Warm, Cold), HUS, MAHA, PNH, PCH, Myelophthisic
Aplastic Acquired PRCA, Diamond-Blackfan anemia, Fanconi anemia, Sideroblastic anemia
Blood tests Normocytic - Microcytic - Macrocytic - Normochromic - Hypochromic
Other Methemoglobinemia
Coagulation/platelets/
coagulopathy/
bleeding diathesis
+
Hypercoagulability primary: Antithrombin III deficiency - Protein C deficiency/Activated protein C resistance/Protein S deficiency/Factor V Leiden - Hyperprothrombinemia
acquired: DIC (Congenital afibrinogenemia, Purpura fulminans) - autoimmune (Antiphospholipid)
Other Essential thrombocytosis
- clotting factor: Hemophilia (A/VIII, B/IX, C/XI) • Von Willebrand disease • Hypoprothrombinemia/II - XIII

platelet function: Bernard-Soulier syndrome - Glanzmann's thrombasthenia - Gray platelet syndrome - May Hegglin anomaly - Pelger-Huet anomaly

Purpura: Henoch-Schönlein, ITP (Evans syndrome), TTP

Thrombocytopenia (Heparin-induced thrombocytopenia)
Monocytes/
macrophages
+
Histiocytosis WHO-I (Langerhans cell histiocytosis)

WHO-II/non-Langerhans-cell (Juvenile xanthogranuloma, Hemophagocytic lymphohistiocytosis)

WHO-III/malignant (Acute monocytic leukemia, Malignant histiocytosis, Erdheim-Chester disease)
Other Chronic granulomatous disease
-cytosis: Monocytosis
- -penia: Monocytopenia
Granulocytes
+ -cytosis: granulocytosis (Neutrophilia, Eosinophilia, Basophilia)
- -penia: Granulocytopenia/agranulocytosis (Neutropenia/Kostmann syndrome, Eosinopenia, Basopenia)
See also hematological malignancy and immune disorders
de:Paroxysmale nächtliche Hämoglobinurieit:Emoglobinuria parossistica notturna

nl:Paroxysmale nocturnale hemoglobinuriesr:Пароксизмална ноћна хемоглобинурија

Retrieved from "http://www.wikidoc.org/index.php?title=Paroxysmal_nocturnal_hemoglobinuria&oldid=787632"
Navigation WikiDoc | WikiPatient | Popular pages | Recently Edited Pages | Recently Added Pictures

Table of Contents In Alphabetical Order | By Individual Diseases | Signs and Symptoms | Physical Examination | Lab Tests | Drugs

Editor Tools Become an Editor | Editors Help Menu | Create a Page | Edit a Page | Upload a Picture or File | Printable version | Permanent link | Maintain Pages | What Pages Link Here
There is no pharmaceutical or device industry support for this site and we need your viewer supported Donations | Editorial Board | Governance | Licensing | Disclaimers | Avoid Plagiarism | Policies
Linked-in.jpg
Personal tools
Namespaces

Variants
Actions
Navigation
Toolbox
In other languages