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Paroxysmal Nocturnal Hemoglobinuria
Matthew T. Heller, MD, FSAR
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Paroxysmal nocturnal hemoglobinuria (PNH)
              • Definitions

                • Rare, acquired stem cell disorder resulting in predisposition to complement-mediated hemolysis

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Ultrasonographic Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      DIAGNOSTIC CHECKLIST

                                      • Image Interpretation Pearls

                                        Selected References

                                        1. Alashkar F et al: The role of whole-body magnetic resonance imaging (WB-MRI) in patients with paroxysmal nocturnal hemoglobinuria (PNH). Sci Rep. 8(1):13458, 2018
                                        2. Park C et al: Paroxysmal nocturnal hemoglobinuria: kidney biopsy and magnetic resonance imaging. Kidney Res Clin Pract. 37(1):96-7, 2018
                                        3. De Cobelli F et al: New insights in abdominal pain in paroxysmal nocturnal hemoglobinuria (PNH): a MRI study. PLoS One. 10(4):e0122832, 2015
                                        4. Brodsky RA: Paroxysmal nocturnal hemoglobinuria. Blood. 124(18):2804-11, 2014
                                        5. Van Bijnen ST et al: Mechanisms and clinical implications of thrombosis in paroxysmal nocturnal hemoglobinuria. J Thromb Haemost. 10(1):1-10, 2012
                                        6. Jain P: An unusual cause of Budd-Chiari syndrome: paroxysmal nocturnal hemoglobinuria. J Gastrointestin Liver Dis. 19(1):107-8, 2010
                                        7. Tufano A et al: Budd-Chiari syndrome in a paroxysmal nocturnal hemoglobinuria patient with previous cerebral venous thrombosis. Intern Emerg Med. 4(1):75-7, 2009
                                        8. Nair RK et al: Spectrum of renal involvement in paroxysmal nocturnal hemoglobinuria: report of three cases and a brief review of the literature. Int Urol Nephrol. 40(2):471-5, 2008
                                        9. Rimola J et al: The kidney in paroxysmal nocturnal haemoglobinuria: MRI findings. Br J Radiol. 77(923):953-6, 2004
                                        10. Jeong JY et al: Atypical low-signal-intensity renal parenchyma: causes and patterns. Radiographics. 22(4):833-46, 2002
                                        11. Titton RL et al: Case 51: paroxysmal nocturnal hemoglobinuria with thrombotic Budd-Chiari syndrome and renal cortical hemosiderin. Radiology. 225(1):67-70, 2002
                                        12. Gayer G et al: Widespread abdominal venous thrombosis in paroxysmal nocturnal hemoglobinuria diagnosed on CT. Abdom Imaging. 26(4):414-9, 2001
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              • Pathology

                                                • Clinical Issues

                                                  • Diagnostic Checklist

                                                    TERMINOLOGY

                                                    • Abbreviations

                                                      • Paroxysmal nocturnal hemoglobinuria (PNH)
                                                    • Definitions

                                                      • Rare, acquired stem cell disorder resulting in predisposition to complement-mediated hemolysis

                                                    IMAGING

                                                    • General Features

                                                      • CT Findings

                                                        • MR Findings

                                                          • Ultrasonographic Findings

                                                            • Imaging Recommendations

                                                              DIFFERENTIAL DIAGNOSIS

                                                                PATHOLOGY

                                                                • General Features

                                                                  • Staging, Grading, & Classification

                                                                    • Microscopic Features

                                                                      CLINICAL ISSUES

                                                                      • Presentation

                                                                        • Demographics

                                                                          • Natural History & Prognosis

                                                                            DIAGNOSTIC CHECKLIST

                                                                            • Image Interpretation Pearls

                                                                              Selected References

                                                                              1. Alashkar F et al: The role of whole-body magnetic resonance imaging (WB-MRI) in patients with paroxysmal nocturnal hemoglobinuria (PNH). Sci Rep. 8(1):13458, 2018
                                                                              2. Park C et al: Paroxysmal nocturnal hemoglobinuria: kidney biopsy and magnetic resonance imaging. Kidney Res Clin Pract. 37(1):96-7, 2018
                                                                              3. De Cobelli F et al: New insights in abdominal pain in paroxysmal nocturnal hemoglobinuria (PNH): a MRI study. PLoS One. 10(4):e0122832, 2015
                                                                              4. Brodsky RA: Paroxysmal nocturnal hemoglobinuria. Blood. 124(18):2804-11, 2014
                                                                              5. Van Bijnen ST et al: Mechanisms and clinical implications of thrombosis in paroxysmal nocturnal hemoglobinuria. J Thromb Haemost. 10(1):1-10, 2012
                                                                              6. Jain P: An unusual cause of Budd-Chiari syndrome: paroxysmal nocturnal hemoglobinuria. J Gastrointestin Liver Dis. 19(1):107-8, 2010
                                                                              7. Tufano A et al: Budd-Chiari syndrome in a paroxysmal nocturnal hemoglobinuria patient with previous cerebral venous thrombosis. Intern Emerg Med. 4(1):75-7, 2009
                                                                              8. Nair RK et al: Spectrum of renal involvement in paroxysmal nocturnal hemoglobinuria: report of three cases and a brief review of the literature. Int Urol Nephrol. 40(2):471-5, 2008
                                                                              9. Rimola J et al: The kidney in paroxysmal nocturnal haemoglobinuria: MRI findings. Br J Radiol. 77(923):953-6, 2004
                                                                              10. Jeong JY et al: Atypical low-signal-intensity renal parenchyma: causes and patterns. Radiographics. 22(4):833-46, 2002
                                                                              11. Titton RL et al: Case 51: paroxysmal nocturnal hemoglobinuria with thrombotic Budd-Chiari syndrome and renal cortical hemosiderin. Radiology. 225(1):67-70, 2002
                                                                              12. Gayer G et al: Widespread abdominal venous thrombosis in paroxysmal nocturnal hemoglobinuria diagnosed on CT. Abdom Imaging. 26(4):414-9, 2001