Osler–Vaquez Disease

Osler–Vaquez disease is an old name for polycythemia vera (PV). PV is a long-lasting blood disease where the bone marrow makes too many red blood cells. Sometimes it also makes too many white blood cells and platelets. Because there are so many cells, the blood becomes thicker (“viscous”). Thick blood flows more slowly and can form clots, which may cause stroke, heart attack, deep-vein thrombosis, or Budd–Chiari syndrome (a clot in the liver veins). PV is a type of myeloproliferative neoplasm (MPN)—a group of bone-marrow cancers where blood-forming cells grow too much. In almost all people with PV, doctors find a change (a mutation) in a gene called JAK2. This change keeps the JAK-STAT signal “switched on” and makes marrow cells grow even when the body does not need them. Merck ManualsPMCWiley Online Library

PV often develops slowly. Some people feel well for years and only learn about it after a routine blood test shows a high hemoglobin/hematocrit (the amount of red cells). Classic hints are itching after a warm shower, red, burning hands or feet (erythromelalgia), headaches, dizziness, and a full feeling in the left upper belly from an enlarged spleen. Merck Manuals+1

Other names

PV has been called by many names in the medical literature:

• Polycythemia vera (PV), primary polycythemia, polycythemia rubra vera, erythremia, Vaquez disease, and Osler–Vaquez disease. Historically, Louis Henri Vaquez first described the disease in 1892, and William Osler added more detailed reports in 1903—hence the older eponym. WikipediaPMC

Types

Doctors may divide PV into practical “types” to guide diagnosis and care:

1. JAK2 V617F–positive PV – the commonest form (the V617F mutation is present in the JAK2 gene). Wiley Online Library

2. JAK2 exon-12–mutated PV – less common; seen in some people who do not have V617F, often with very high red cells but near-normal platelets and white cells. PMC

3. Masked PV – early PV where hemoglobin/hematocrit are not very high (sometimes due to iron deficiency), but other criteria (JAK2 mutation, bone-marrow pattern, low erythropoietin) fit PV. Wiley Online Library

4. Overt PV – classic, obvious high hemoglobin/hematocrit with the full set of blood and marrow features. Wiley Online Library

Causes

PV is not caused by smoking or low oxygen (those cause secondary polycythemia). PV is a primary, clonal bone-marrow disease. Below are 20 biological drivers and contributors that explain why PV happens and why it behaves the way it does:

1. JAK2 V617F mutation – the key driver in the vast majority of PV cases; it makes signaling for growth stay “on” even without erythropoietin (EPO). Wiley Online Library

2. JAK2 exon-12 mutations – found in a smaller group without V617F; these also keep JAK-STAT signaling active. PMC

3. Constitutive JAK-STAT pathway activation – the downstream effect of JAK2 mutations; marrow precursors grow and survive too much. Merck Manuals

4. EPO-independent erythroid colony growth – red-cell precursors multiply without EPO stimulation because of mutant signaling. Wiley Online Library

5. Panmyelosis in the marrow – not only red cells but also white cells and platelets expand, adding to viscosity and clot risk. Wiley Online Library

6. Low serum erythropoietin (feedback effect) – the kidney reduces EPO because the body senses too many red cells; this lab clue supports a primary process. Wiley Online Library

7. Inherited predisposition (JAK2 46/1 haplotype) – a common DNA background that makes JAK2 mutations more likely to arise in some people. PubMed

8. Additional somatic mutations (e.g., TET2, ASXL1, DNMT3A) – cooperating gene changes can influence progression and complications. Wiley Online Library

9. Higher JAK2 mutant allele burden – a larger proportion of mutated cells is linked with more symptoms and higher risks (e.g., thrombosis). Ash Publications

10. Age-related clonal hematopoiesis – with age, marrow clones with growth advantages become more common, increasing PV risk. Wiley Online Library

11. Pro-thrombotic blood rheology – high hematocrit thickens blood and alters shear stress, which promotes clot formation. Merck Manuals

12. Platelet activation and leukocytosis – overactive platelets and high white cells add to clotting and inflammation. Merck Manuals

13. Marrow micro-environment changes – supporting cells and cytokines favor the mutant clone’s survival and growth. Wiley Online Library

14. Chronic inflammation – PV can create a cycle of inflammation that further supports the malignant clone. Wiley Online Library

15. Basophil/mast cell mediator release – contributes to intense itching (especially after warm water) and flushing. Merck Manuals

16. Uric-acid overproduction – fast cell turnover makes excess uric acid, which can cause gout and kidney stones. Merck Manuals

17. Relative iron deficiency – rapid red-cell making uses iron; low iron can “mask” high hemoglobin and delay recognition. Wiley Online Library

18. Splenic sequestration and enlargement – the spleen works hard filtering excess cells and often enlarges, adding symptoms like early fullness. Merck Manuals

19. Microvascular dysfunction – clogged tiny vessels cause burning pain in hands/feet and visual disturbances. Merck Manuals

20. Natural disease evolution – over time PV may shift toward myelofibrosis or rarely acute leukemia through extra mutations. Merck Manuals

Symptoms

1. No symptoms at first – many people feel fine; the problem is first seen on a routine blood test. Merck Manuals

2. Headache and dizziness – from thick blood and reduced flow in small vessels. Merck Manuals

3. Itching after a warm bath or shower (aquagenic pruritus) – very common and troublesome in PV. Merck Manuals

4. Burning, red hands or feet (erythromelalgia) – micro-vessel blockages cause heat, redness, and pain. Merck Manuals

5. Tiredness and weakness – common in many blood diseases and in PV. Merck Manuals

6. Visual symptoms (blurry vision, flashing lights, blind spots) – due to microvascular spasm or clotting. Merck Manuals

7. Ringing in the ears or fullness in the head – from high blood thickness. Merck Manuals

8. Shortness of breath or chest pain – can be from anemia-like symptoms of poor flow or from clots. Merck Manuals

9. Nosebleeds or easy bruising – paradoxical bleeding can happen even while clot risk is high. Merck Manuals

10. Red or flushed face (plethora) – visible sign of too many red cells. Merck Manuals

11. Enlarged spleen (left-upper-belly fullness, early satiety) – common in PV. Merck Manuals

12. Gout (painful big toe or other joints) – from high uric acid. Merck Manuals

13. Numbness or tingling in fingers/toes – reduced microcirculation. Merck Manuals

14. Night sweats and weight loss – constitutional symptoms some patients report. Merck Manuals

15. Clot events – stroke symptoms, leg swelling/pain (DVT), sudden shortness of breath (PE), or liver-vein clot (Budd–Chiari). Merck Manuals

Diagnostic tests

A) Physical examination (doctor looks, listens, and feels)

1. General inspection for facial redness (plethora) – a red, ruddy face suggests many red cells. The doctor also notes scratching marks from itching. Merck Manuals

2. Vital signs and blood pressure – high blood pressure can occur with thicker blood; pulse may be bounding. Merck Manuals

3. Spleen and liver check – the doctor palpates the left upper abdomen for an enlarged spleen and the right side for the liver. Splenomegaly is common in PV. Merck Manuals

4. Skin, hands, and feet – red, hot, painful hands/feet (erythromelalgia) point toward PV-type microvascular problems. Merck Manuals

5. Neurologic screen – brief bedside checks for weakness, speech problems, or vision loss to look for possible past or current clots. Merck Manuals

B) Manual tests (simple bedside maneuvers done by hand)

6. Abdominal palpation and percussion for spleen (e.g., Castell’s sign) – a hands-on way to detect splenic enlargement. Merck Manuals

7. Capillary refill time (pressing a fingertip and timing color return) – slow refill can reflect microcirculation problems in thick blood. Merck Manuals

8. Peripheral pulse and temperature comparison (hands/feet) – asymmetry or heat/redness supports erythromelalgia from PV. Merck Manuals

C) Laboratory and pathological tests

9. Complete blood count (CBC) – shows high hemoglobin/hematocrit; many people also have high white cells and platelets. The WHO diagnostic thresholds used in practice are Hb >16.5 g/dL (men) or >16.0 g/dL (women), or hematocrit >49% (men) or >48% (women), together with other criteria. PMCWiley Online Library

10. Serum erythropoietin (EPO) level – low in PV (the kidney turns EPO down because red cells are already high). Wiley Online Library

11. JAK2 mutation testing – first test is JAK2 V617F; if negative and suspicion remains, test JAK2 exon-12. Nearly all PV patients carry one of these. Wiley Online LibraryPMC

12. Bone-marrow biopsy/aspirate – shows hypercellular marrow with panmyelosis (too many erythroid, granulocytic, and megakaryocytic cells) and helps exclude other diseases. Wiley Online Library

13. Arterial blood gas or oxygen saturation (to rule out secondary causes) – PV usually has normal oxygen; low oxygen suggests a secondary cause. Merck Manuals

14. Uric acid, LDH, and iron studies (ferritin, transferrin saturation) – high uric acid/LDH reflect cell turnover; iron may be low because the marrow uses it quickly. Merck ManualsWiley Online Library

Putting the above together, modern criteria (widely used in practice) diagnose PV when all three major criteria are met—(1) high hemoglobin/hematocrit or increased red-cell mass, (2) panmyelosis on marrow biopsy, and (3) a JAK2 mutation—or the first two major plus the minor criterion (subnormal EPO). PMCWiley Online Library

D) Electrodiagnostic tests (electronic physiologic tests)

15. Electrocardiogram (ECG) – looks for heart strain or evidence of past ischemia that can occur with clots. Merck Manuals

16. Pulse oximetry – a quick sensor on the finger that checks oxygen level; normal oxygen supports PV (primary) rather than a low-oxygen secondary cause. Merck Manuals

E) Imaging tests

17. Abdominal ultrasound – measures spleen size and checks the liver; spleen enlargement is common in PV. Merck Manuals

18. Doppler ultrasound of veins – looks for deep-vein thrombosis (DVT) in the legs if there is swelling or pain. Merck Manuals

19. CT pulmonary angiography – checks for pulmonary embolism (PE) if there is sudden shortness of breath or chest pain. Merck Manuals

20. MRI or CT of the brain – used when stroke-like symptoms occur to confirm a clot or small-vessel event. Merck Manuals

Non-pharmacological treatments

(I group them so they’re easy to follow. “Purpose” explains why; “Mechanism/Benefits” explain how they help.)

A) Core medical procedures and routines (most proven)

1. Therapeutic phlebotomy (blood removal).
   Purpose: Quickly lower hematocrit under 45%.
   Mechanism/Benefits: Removing ~250–500 mL of blood reduces red cell mass and blood thickness; often weekly at first, then less often. This is the cornerstone of PV care. AetnaAAFP

2. Regular monitoring plan.
   Purpose: Catch changes early.
   Mechanism/Benefits: Check CBC, iron status, symptoms, and clot/bleed risks regularly; keeps hematocrit on target and guides dosing of any medicines. AAFP

3. Avoiding unnecessary iron supplements.
   Purpose: Prevent fueling extra red cell production.
   Mechanism/Benefits: Iron can speed RBC production in PV; supplementation is usually avoided unless severe, symptomatic deficiency exists and is closely monitored. PMCPubMedAdvanced Practitioner Resources

4. Vaccinations (flu, pneumococcal, COVID-19 per local guidance).
   Purpose: Prevent infections that can trigger complications or hospital stays.
   Mechanism/Benefits: Lowers systemic stress and inflammation that can worsen symptoms or clot risk (standard adult vaccine best-practice). (General preventive care recommendation.)

5. Smoking cessation.
   Purpose: Reduce clot risk and blood vessel damage.
   Mechanism/Benefits: Smoking thickens blood and damages endothelium; stopping reduces events (strong cardiovascular prevention principle).

6. Heart-risk control (BP, cholesterol, diabetes).
   Purpose: Lower overall clot risk.
   Mechanism/Benefits: Managing these risks reduces arterial events alongside PV-specific care. (Guideline principle in PV management.) Targeted Oncology

B) Physiotherapy / physical measures

7. Hydration coaching.
   Purpose: Keep blood less viscous day-to-day.
   Mechanism/Benefits: Adequate fluids improve plasma volume and flow (symptom relief).

8. Graded aerobic exercise (walking, cycling).
   Purpose: Improve circulation, weight, mood.
   Mechanism/Benefits: Better endothelial function, lower inflammation, supports weight and BP control (clot-risk reduction).

9. Leg movement and calf-pump drills during long sitting.
   Purpose: Prevent leg vein stasis.
   Mechanism/Benefits: Muscle pumping keeps blood moving; reduces DVT risk during travel.

10. Compression stockings on long flights if advised.
    Purpose: Reduce venous stasis.
    Mechanism/Benefits: External pressure supports venous return (travel thrombosis prevention).

11. Cool-water showering and brief warm exposure.
    Purpose: Reduce water-triggered itch (aquagenic pruritus).
    Mechanism/Benefits: Heat can trigger mast cell mediators and itch; cooler water and short showers lower triggers. (Pruritus is very common in PV.) AAFP

12. Skin emollients and post-bath pat-dry technique.
    Purpose: Reduce itch and micro-tears.
    Mechanism/Benefits: Strong moisturizers help the skin barrier and reduce itch cycles.

13. Narrow-band UVB (NB-UVB) phototherapy when itch is severe and drugs fail (done by dermatology).
    Purpose: Relieve refractory pruritus.
    Mechanism/Benefits: NB-UVB can ease PV-related itch within weeks; evidence supports benefit. PubMed

14. Hand/foot cooling or elevation during erythromelalgia flares.
    Purpose: Relieve burning/redness in hands/feet.
    Mechanism/Benefits: Local cooling and elevation reduce small-vessel dilation and symptoms (aspirin helps too—see drugs). Wiley Online Library

15. Balance and fall-prevention exercises (if older or dizzy).
    Purpose: Reduce head-injury bleeding risk if on aspirin/anticoagulants.
    Mechanism/Benefits: Safer mobility lowers harm from potential falls.

16. Sleep hygiene and treatment of sleep apnea if present.
    Purpose: Limit hypoxia-driven symptoms and fatigue.
    Mechanism/Benefits: Better oxygenation and rest reduce headaches and daytime sleepiness.

17. Weight management plan (diet + activity).
    Purpose: Lower clot risk and BP, improve mobility.
    Mechanism/Benefits: Reduces inflammatory burden and venous stasis.

18. Heat-trigger avoidance plan.
    Purpose: Reduce itch/headache flares.
    Mechanism/Benefits: Hot tubs/saunas can worsen aquagenic pruritus and vasodilation.

19. Travel safety checklist (move every hour, hydrate, compression, aisle seat).
    Purpose: Prevent travel-related DVT.
    Mechanism/Benefits: Combines multiple small protections into one routine.

C) Mind-body & educational therapy

20. Mindfulness/relaxation/slow breathing.
    Purpose: Reduce the stress-itch cycle.
    Mechanism/Benefits: Calms sympathetic arousal that can amplify itch and pain perception.

21. Cognitive-behavioral strategies for chronic itch/pain.
    Purpose: Cut the scratch–itch loop.
    Mechanism/Benefits: Skills to redirect attention and break habits that worsen skin irritation.

22. PV self-management teaching.
    Purpose: Understand targets (Hct <45%), triggers, meds, and warning signs.
    Mechanism/Benefits: Informed patients stay on target and seek help sooner. NCCN

23. Medication adherence training (pill boxes/phone prompts).
    Purpose: Ensure steady aspirin or cytoreductive dosing.
    Mechanism/Benefits: Steady therapy prevents breakthrough events.

24. Safe-exercise counseling (with count-based adjustments).
    Purpose: Stay active without over-straining if counts are very high or very low.
    Mechanism/Benefits: Tailors activity to symptoms and lab values.

25. About “gene therapy.”
    Purpose: Set expectations.
    Mechanism/Benefits: There is no approved gene therapy for PV today. Research is ongoing, but current standard care uses phlebotomy, aspirin, and (when needed) cytoreduction or JAK-inhibitors/interferon. (This keeps care realistic.) Targeted Oncology

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Drug treatments

(Doses are typical ranges—your hematologist personalizes them. Do not start/stop anything without your clinician.)

1. Aspirin, low dose (antiplatelet).
   Dose/Time: 75–100 mg once daily (often 81 mg).
   Purpose: Lower clot risk; help erythromelalgia.
   Mechanism: Blocks platelet thromboxane A2.
   Side effects: Upset stomach, bleeding risk. New England Journal of Medicine

2. Hydroxyurea (cytoreductive antimetabolite).
   Dose/Time: Commonly 500–1000 mg/day (or ~15–20 mg/kg/day) adjusted to counts.
   Purpose: First-line cytoreduction in many high-risk adults.
   Mechanism: Slows DNA synthesis in marrow cells.
   Side effects: Low counts, mouth ulcers, skin/nail changes; rare leg ulcers. Drugs.comTargeted Oncology

3. Ropeginterferon alfa-2b (BESREMi) (interferon, disease-modifying).
   Dose/Time: Start 100 mcg every 2 weeks (50 mcg if on hydroxyurea), increase by 50 mcg q2w to max 500 mcg; later may extend to every 4 weeks once stable.
   Purpose: Cytoreduction with potential molecular responses; suitable across risk groups.
   Mechanism: Immune/antiproliferative signaling that quiets the malignant clone.
   Side effects: Flu-like symptoms, mood changes, liver tests up, thyroid issues (monitoring needed). FDA Access DataPA Health & Wellness

4. Peginterferon alfa-2a (off-label in PV; weekly interferon).
   Dose/Time: Often 45–90 mcg once weekly, titrating up (max ~180 mcg weekly) based on response/tolerance.
   Purpose: Alternative cytoreduction, including in younger patients and pregnancy planning.
   Mechanism: Similar to above.
   Side effects: As above; monitoring is key. HSE.iePMC

5. Ruxolitinib (JAK1/2 inhibitor).
   Dose/Time: 10 mg twice daily to start in PV after hydroxyurea failure/intolerance; adjust with counts (max 25 mg BID).
   Purpose: Control hematocrit without phlebotomy, shrink spleen, ease symptoms/itch.
   Mechanism: Blocks overactive JAK signaling from the JAK2 mutation.
   Side effects: Anemia, low platelets, infections (monitor CBC). Avoid strong CYP3A4 inhibitors/large grapefruit intake. hcp.jakafi.com

6. Busulfan (alkylator; for selected older/intolerant patients).
   Dose/Time: Very low-dose chronic schedules such as 2 mg/day or about 14 mg/week, titrated to response, used sparingly.
   Purpose: Second/third-line cytoreduction when other options fail.
   Mechanism: DNA cross-linking to suppress marrow.
   Side effects: Cytopenias, longer-term leukemic risk with cumulative exposure—specialist supervision only. Ash PublicationsPMC

7. Antihistamines (H1 blockers like cetirizine).
   Dose/Time: Standard daily doses.
   Purpose: Reduce itching.
   Mechanism: Blocks histamine signaling.
   Side effects: Drowsiness (older agents), dry mouth. AAFP

8. SSRI for itch (paroxetine).
   Dose/Time: Common starting 10–20 mg daily; titrate per clinician.
   Purpose: Refractory pruritus relief.
   Mechanism: Central itch modulation.
   Side effects: Nausea, sexual dysfunction, sleep changes. AAFP

9. Gabapentin / Pregabalin (neuromodulators for pruritus).
   Dose/Time: Gabapentin often 100–300 mg at night to start; adjust slowly.
   Purpose: Help nerve-mediated chronic itch.
   Mechanism: Reduces neuronal excitability.
   Side effects: Sleepiness, dizziness. PMC

10. Allopurinol (urate-lowering).
    Dose/Time: Often start 100 mg daily, titrate to uric acid goal if gout/high urate.
    Purpose: Prevent gout flares from high cell turnover.
    Mechanism: Blocks xanthine oxidase.
    Side effects: Rash (rare severe), liver tests; dose-adjust in CKD. PMC

11. Anticoagulants (DOACs or warfarin) when a clot occurs.
    Dose/Time: Standard VTE dosing per agent.
    Purpose: Treat/secondary-prevent PV-related DVT/PE or splanchnic vein thrombosis.
    Mechanism: Prevents new clot extension.
    Side effects: Bleeding—specialist follows closely. (General VTE practice; PV often linked with hepatic vein clots—Budd-Chiari). ScienceDirect

12. Topical menthol/pramoxine or capsaicin for localized itch (as adjuncts).
    Dose/Time: Per product label.
    Purpose: Local itch relief.
    Mechanism: Counter-stimulation of itch fibers.
    Side effects: Mild burning/irritation.

13. Proton-pump inhibitor or H2 blocker if aspirin causes dyspepsia (doctor decides).
    Purpose: Protect stomach so aspirin can be continued.
    Mechanism: Reduces acid and bleeding risk in select patients.
    Side effects: Vary by agent.

14. Cytoreduction with hydroxyurea + aspirin combo (when indicated).
    Purpose: Standard high-risk backbone (not a new drug, but a common combined plan).
    Mechanism/Side effects: As above. AAFP

15. Interferon (ropeg or peg) as first-line cytoreduction (especially younger pts or pregnancy planning).
    Purpose: Disease-modifying potential; can reduce JAK2 allele burden over time.
    Mechanism: Immune and anti-proliferative effects.
    Side effects: Flu-like symptoms, mood/thyroid effects (monitor). Wiley Online Library

Important note on “immune-booster/regenerative/stem-cell drugs”:
There are no approved “stem-cell drugs” to regenerate marrow for PV. Allogeneic stem-cell transplant is a procedure (see below), not a pill. Interferons are the main disease-modifying drugs. Ruxolitinib blocks the overactive JAK pathway. Investigational agents like rusfertide (a hepcidin mimetic) and givinostat (HDAC inhibitor) are being studied; they are not standard outside trials. Taylor & Francis Online

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Dietary molecular supplements

PV does not have proven supplement cures. Food-first is best. Always clear supplements with your hematologist—some can raise bleeding risk (with aspirin) or interact with drugs.

1. Omega-3 fatty acids (EPA/DHA).
   Dose: ~1 g/day combined EPA+DHA from food/supplement if your clinician agrees.
   Function/Mechanism: May mildly reduce platelet reactivity and inflammation; general heart health. Office of Dietary Supplements

2. Vitamin D (if low).
   Dose: Commonly 800–2000 IU/day, individualized to blood levels.
   Function: Bone/immune support; corrects deficiency common in adults. Office of Dietary Supplements

3. Folate from diet or low-dose supplement (if deficient).
   Function: Normal RBC formation; correct deficiency if present (but do not use to “treat PV”).

4. Magnesium (diet-first; supplement only if low).
   Function: Muscle/nerve function; may help sleep.

5. CoQ10 (optional).
   Function: General mitochondrial support; evidence for PV is minimal—discuss first.

6. Vitamin B12 (only if low).
   Function: Nerve and blood health; correct deficiency if proven.

7. Niacinamide (not niacin) small doses if advised.
   Function: Skin barrier/energy pathways; avoid high-dose niacin (flushing can worsen symptoms).

8. Turmeric/curcumin (caution with bleeding).
   Function: Anti-inflammatory; may increase bleeding risk with aspirin—doctor approval needed.

9. Probiotics/fermented foods (food-first).
   Function: Gut comfort if on meds that upset stomach.

10. Electrolyte solutions for hydration on hot days/travel.
    Function: Supports fluid balance; avoids dehydration, a clot trigger.

Avoid starting iron or high-iron multivitamins on your own in PV; this can increase red cell production. If iron deficiency causes severe symptoms, any supplementation is done very carefully with close blood monitoring. PMCAdvanced Practitioner Resources

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Regenerative / stem-cell” drugs

1. Ropeginterferon alfa-2b – immune-modulating, disease-modifying (approved). (See dosing above.) FDA Access Data

2. Peginterferon alfa-2a – weekly, off-label in PV, disease-modifying potential. HSE.ie

3. Ruxolitinib – targeted JAK inhibitor; relieves symptoms and controls hematocrit. hcp.jakafi.com

4. Rusfertide (PTG-300, investigational) – a hepcidin mimetic under study; reduced phlebotomy needs in trials. Not approved. Taylor & Francis Online

5. Givinostat (investigational HDAC inhibitor) – studied in PV; not standard. (Investigational context.)

6. No approved “regenerative stem-cell drug.” The only curative approach is allogeneic stem-cell transplantation, a procedure for select advanced cases (see below).

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Surgeries/procedures

1. Therapeutic phlebotomy (procedure, not an operating-room surgery).
   Why: Fastest way to bring hematocrit under 45% early and for maintenance. Aetna

2. Red-cell apheresis (erythrocytapheresis).
   Why: Rapid reduction of red cells in urgent situations (specialist centers).

3. Allogeneic hematopoietic stem-cell transplantation (HSCT).
   Why: Potentially curative, but high risk; reserved for rare cases with progression (e.g., to myelofibrosis/AML) or uncontrolled disease after all standard options.

4. Splenectomy (rare).
   Why: For massive, painful spleen with repeated problems despite medicines; done less often today because medical options improved.

5. TIPS (transjugular intrahepatic portosystemic shunt) or other portal procedures for Budd-Chiari syndrome (hepatic vein clots) when needed.
   Why: Decompress liver veins and manage portal hypertension in PV-related splanchnic vein thrombosis—done by interventional radiology/hepatology. ScienceDirect

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Prevention habits

1. Keep hematocrit <45% with your care plan. AAFP

2. Take low-dose aspirin daily unless your doctor says not to. New England Journal of Medicine

3. Don’t smoke; avoid secondhand smoke.

4. Hydrate well, especially in heat and on travel days.

5. Move every hour on long trips; consider compression stockings.

6. Control blood pressure, cholesterol, and blood sugar.

7. Healthy weight and regular activity.

8. Avoid iron supplements unless your team prescribes them. PMC

9. Limit very hot baths/saunas if they trigger itch or flushing.

10. Keep vaccines updated and get medical clearance before starting new over-the-counter supplements.

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When to see a doctor urgently

• New chest pain, severe shortness of breath, sudden weakness/speech trouble, one-sided body changes, or severe headache (possible clot/stroke).

• New leg swelling/pain (possible DVT) or belly pain/swelling (possible splanchnic clot).

• Bleeding you cannot explain, black stools, or bruising with minimal injury.

• Fever, night sweats, unintentional weight loss, rapidly enlarging spleen area (left upper belly), or severe, persistent itch.

• You are pregnant or planning pregnancy (PV plans change during pregnancy).

• Hematocrit trending above 45% despite treatment. AAFP

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What to eat and what to avoid

Eat more of:

• Water and unsweetened fluids through the day.

• Whole foods: vegetables, fruits, beans, whole grains.

• Fish rich in omega-3 (e.g., sardines, salmon) once or twice weekly—heart health. Office of Dietary Supplements

• Healthy fats (olive oil, nuts in modest amounts).

• High-fiber foods to support gut and stool regularity if taking constipating meds.

Limit/avoid:

• Alcohol excess, especially if you’ve had liver vein clots.

• Dehydrating drinks in large amounts (e.g., heavy caffeine without water).

• Iron supplements or high-iron multivitamins unless your hematologist prescribes them. PMC

• Herbal products that increase bleeding risk (e.g., high-dose turmeric) if you’re on aspirin/anticoagulants—discuss first.

• Grapefruit/strong CYP3A4 inhibitors if you take ruxolitinib (dose interactions). hcp.jakafi.com

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FAQs

1) Is Osler-Vaquez disease the same as polycythemia vera?
Yes. It’s an older name for PV. The modern term is PV.

2) What number should I remember for safety?
Keep hematocrit under 45%. That number lowers clot risks. AAFP

3) Will I need phlebotomy forever?
Often at first, then less often once counts stabilize or a cytoreductive medicine is added. Aetna

4) Do most people with PV have a JAK2 mutation?
Yes—about 95% carry JAK2 V617F; many of the rest have JAK2 exon-12 mutations. Nature

5) Why do I itch after a shower?
That’s aquagenic pruritus, common in PV. Cooler water, antihistamines, SSRIs, ruxolitinib, or NB-UVB phototherapy can help. AAFPPubMed

6) Is aspirin safe for everyone?
Not for people with active bleeding or very low platelets or aspirin allergy—your doctor will decide. New England Journal of Medicine

7) Do I need a “blood thinner” too?
Only if you have a clot or certain high-risk situations; otherwise aspirin is usually enough. (Doctor decides.) ScienceDirect

8) Can I take iron for fatigue?
Usually no in PV, unless your team finds severe, symptomatic deficiency and closely monitors you. PMC

9) Which drug lowers counts first-line?
Many adults use hydroxyurea; interferon (especially ropeginterferon) is increasingly used and can be first-line too. Wiley Online Library

10) What if hydroxyurea doesn’t work or I can’t tolerate it?
Ruxolitinib is approved for hydroxyurea-resistant/intolerant PV and helps symptoms and spleen. hcp.jakafi.com

11) Are there new treatments coming?
Yes. Rusfertide (hepcidin mimetic) and other agents are under study to reduce phlebotomy needs and control counts. Taylor & Francis Online

12) Can PV cause belly-vein clots like Budd-Chiari?
Yes, PV and other JAK2-positive states are common causes of splanchnic vein thrombosis. ScienceDirect

13) When is stem-cell transplant used?
Rarely—reserved for advanced disease or transformation when risks of other options outweigh transplant risks.

14) Can I live a long life with PV?
Many people do very well for years with good control of hematocrit, aspirin use, and risk-factor management. (Prognosis varies by age, events, and response.)

15) What’s my day-to-day checklist?
Hydrate, move often, take medicines as prescribed, track hematocrit, avoid unapproved supplements (especially iron), and call your team for any warning signs

Disclaimer: Each person’s journey is unique, treatment plan, life style, food habit, hormonal condition, immune system, chronic disease condition, geological location, weather and previous medical  history is also unique. So always seek the best advice from a qualified medical professional or health care provider before trying any treatments to ensure to find out the best plan for you. This guide is for general information and educational purposes only. Regular check-ups and awareness can help to manage and prevent complications associated with these diseases conditions. If you or someone are suffering from this disease condition bookmark this website or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. We always try to ensure that the content is regularly updated to reflect the latest medical research and treatment options. Thank you for giving your valuable time to read the article.

The article is written by Team RxHarun and reviewed by the Rx Editorial Board Members

Last Updated: September 02, 2025.

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