Polycythemia classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Debduti Mukhopadhyay, M.B.B.S[2]

Overview

There is no established system for the classification of [disease name].

OR

[Disease name] may be classified according to [classification method] into [number] subtypes/groups: [group1], [group2], [group3], and [group4].

OR

[Disease name] may be classified into [large number > 6] subtypes based on [classification method 1], [classification method 2], and [classification method 3]. [Disease name] may be classified into several subtypes based on [classification method 1], [classification method 2], and [classification method 3].

OR

Based on the duration of symptoms, [disease name] may be classified as either acute or chronic.

OR

If the staging system involves specific and characteristic findings and features: According to the [staging system + reference], there are [number] stages of [malignancy name] based on the [finding1], [finding2], and [finding3]. Each stage is assigned a [letter/number1] and a [letter/number2] that designate the [feature1] and [feature2].

OR

The staging of [malignancy name] is based on the [staging system].

OR

There is no established system for the staging of [malignancy name].

Classification

Once the diagnosis of absolute erythrocytosis has been established, red cell mass (RCM > 125% of predicted), classification can be done accordingly :

• Primary erythrocytosis

   - Polycythemia vera

• Secondary erythrocytosis

   - Congenital:
         - Erythropoietin receptor-mediated
         - High oxygen affinity hemoglobin
         - Bisphosphoglycerate mutase deficiency  
         - VHL (Von Hippel-Lindau) gene mutation (Chuvash erythrocytosis)
         - PHD2 mutations
         - HIF-2 alpha mutations
   - Acquired:
         - Hypoxia driven
             - Central hypoxic process:
                 - Chronic Lung disease
                 - Right-to-left cardiopulmonary vascular shunts
                 - Carbon monoxide poisoning
                 - Smoker's erythrocytosis
                 - Hypoventilation syndromes including sleep apnea (high-altitude habitat)
             - Local renal hypoxia:
                 - Renal Artery Stenosis
                 - End Stage Renal Disease
                 - Hydronephrosis
                 - Renal cysts (polycystic kidney disease)
                 - Postrenal transplant erythrocytosis
             - Pathologic EPO production:
                 - Tumors
                    - Cerebellar hemangioblastoma
                    - Meningioma
                    - Parathyroid carcinoma/adenomas
                    - Hepatocellular carcinoma
                    - Renal cell cancer
                    - Pheochromocytoma
                    - Uterine leiomyomas
              - Exogenous EPO:
                  - Drug associated 
                    - EPO administration
                    - Androgen administration
    - Idiopathic erythrocytosis