Paroxysmal Nocturnal Hemoglobinuria (PNH) (Marchiafava–Micheli Syndrome)




Ham Acid Lysis Test

Sucrose Lysis Test

Thrombin Lysis Test

Flow Cytometry of Peripheral Blood with CD55 + CD59 Antibodies

Flow Cytometry of Peripheral Blood with CD59/CD24/CD16 or Any Other GPI-Linked Proteins Expressed on Granulocytes

Flow Cytometry with Fluourescently Labelled Inactive Toxin Aerolysin (FLAER)

PIGA Gene Mutation Analysis

Clinical Manifestations (Classical Clinical Triad of Complement-Mediated Intravascular Hemolytic Anemia + Thrombosis + Bone Marrow Failure)

General Features

Hematologic Manifestations

Hypercoagulable State (see Hypercoagulable States, [[Hypercoagulable States]])

Anemia/Hemolytic Anemia (see Anemia, [[Anemia]] and Hemolytic Anemia, [[Hemolytic Anemia]])

Thrombocytopenia (see Thrombocytopenia, [[Thrombocytopenia]])

Aplastic Anemia/Pancytopenia (see Pancytopenia, [[Pancytopenia]])

Acute Myeloid Leukemia (AML) (see Acute Myeloid Leukemia, [[Acute Myeloid Leukemia]])

Renal Manifestations

Gastrointestinal Manifestations

Recurrent Abdominal Pain (see Abdominal Pain, [[Abdominal Pain]])

Dysphagia (see Dysphagia, [[Dysphagia]])

Budd-Chiari Syndrome/Hepatic Vein Thrombosis (see Hepatic Vein Thrombosis, [[Hepatic Vein Thrombosis]])

Other Manifestations


Transfusion of Filtered Packed Red Blood Cells (PRBC) (see Packed Red Blood Cells, [[Packed Red Blood Cells]])

Folate/Iron Supplementation (see Folate, [[Folate]] and Iron, [[Iron]])

Prednisone (see Prednisone, [[Prednisone]])


Eculizumab (Soliris) (see Eculizumab, [[Eculizumab]])

Allogeneic Bone Marrow Transplant (BMT)/Stem Cell Transplant (SCT) (see Bone Marrow Transplant, [[Bone Marrow Transplant]])

Anti-Thymocyte Globulin (ATG) or Cyclosporine A (see Anti-Thymocyte Globulin, [[Anti-Thymocyte Globulin]] and Cyclosporine A, [[Cyclosporine A]])