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Polycythemia (Erythrocytosis), Polycythemia Rubra Vera, and Secondary Polycythemia

August 29, 2024HematologyLab Tests

Table of Contents

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  • Polycythemia (Erythrocytosis)
        • What sample is needed to study the cause of Polycythemia?
        • How will you define Polycythemia?
        • What are the Criteria for labeling polycythemia?
        • How would you classify Polycythemia?
        • What is the importance of Polycythemia?
  • Polycythemia Rubra Vera (primary)
        • What are the important facts about Polycythemia vera?
        • What is the onset of polycythemia Rubra Vera?
        • What is the presentation of polycythemia rubra vera?
        • What are the lab findings of Polycythemia Rubra Vera?
        • What are the causes of polycythemia (Rubra) vera?
        • What are the signs and Symptoms of Polycythemia (Rubra) vera?
        • How will you diagnose Polycythemia (Rubra) Vera?
        • How will you treat polycythemia Rubra Ver?
        • What will the prognosis be for polycythemia Rubra Vera?
  • Secondary polycythemia
        • What are the causes of Causes of secondary polycythemia?
        • How will you diagnose Secondary polycythemia?
  • Relative polycythemia (Pseudopolycythemia)
        • What are the laboratory findings of Relative Polycythemia?
        • How will you treat the Polycythemia?
        • How will you summarize various Polycythemias?
      • questions and answers:

Polycythemia (Erythrocytosis)

What sample is needed to study the cause of Polycythemia?

  • EDTA blood sample is required.

How will you define Polycythemia?

  1. Polycythemia is an increase in the total blood RBCs, more than the normal upper limit.
  2. There is increased hemoglobin and hematocrit.
  3. This is an uncontrolled proliferation of red blood cells.
    1. There is increased hemoglobin concentration above the normal upper limit.
Polycythemia

Polycythemia (Erythrocytosis)

What are the Criteria for labeling polycythemia?

  1. RBCs = count increased, more than the normal upper limit.
  2. PCV = Increased.
  3. Hemoglobin = Increased >18 g/dL (180 g/L).
    1. Male  = 18 g/dL (170 g/L)
    2. Female  = 16 g/dL (160 g/L).
  4. Hematocrit = Increased.
    1. Male = >55%
    2. Female = >50%

How would you classify Polycythemia?

  1. Types of  Polycythemia. There are three types :
    1. Primary polycythemia (also known as polycythemia rubra vera).
    2. Secondary polycythemia.
    3. Relative polycythemia.
  2. There is pseudopolycythemia, where red cell volume is normal, but the plasma volume is reduced.
         Parameters Normal value  Relative Polycythemia Primary/secondary polycythemia
  • Plasma volume
  • 40 to 50 mL/Kg
  • Decreased
  • Normal
  • Total red blood cell volume
  1. Male = 25 to 35 mL/Kg
  2. Female = 22 to 32 mL/Kg
  • Normal
  • Increased

What is the importance of Polycythemia?

  1. There is hyperviscosity.
  2. In primary polycythemia, there is an uncontrolled clonal proliferation of stem cells.
  3. A chromosomal abnormality of the deletion of 9p or 20q was found in the minority of the cases.

Polycythemia Rubra Vera (primary)

  • Polycythemia vera is also called polycythemia rubra vera.

What are the important facts about Polycythemia vera?

  1. This is a bone marrow disorder where the bone marrow produces an uncontrolled RBC proliferation.
    1. This is basically a myeloproliferative disorder.
    2. This is a stem cell disorder.
  2. Erythrocytosis is not dependent on erythropoietin.

What is the onset of polycythemia Rubra Vera?

  1. The median age at diagnosis is 60 years. It occurs between 40 to 70m years of age.
  2. This is more common in men than in women.

What is the presentation of polycythemia rubra vera?

  1. This is a chronic disease; many patients die from thrombosis or cardiac arrest.
  2. Polycythemia vera may also produce too many other types of blood cells, such as white blood cells and platelets.
  3. Polycythemia vera is also called primary polycythemia.
  4. It is rare and usually develops slowly.
  5. Polycythemia vera is often found during a blood test for some other reason.
  6. These patients who survive long may develop myelofibrosis or myeloid leukemia.

What are the lab findings of Polycythemia Rubra Vera?

  1. Laboratory findings are:
    1. Increased RBC count, Hb, and Hct.
    2. Normal MCV and MCH.
    3. Increased WBCs and platelets.
  2. Bone marrow shows panhyperplasia.

What are the causes of polycythemia (Rubra) vera?

  1. Unknown.
  2. Bone marrow abnormality
  3. 95 % of the cases have a genetic abnormality.

What are the signs and Symptoms of Polycythemia (Rubra) vera?

  1. This is often found incidentally when raised hemoglobin and RBC counts are found.
  2. This is the disease of old age with equal sex distribution.
  3. Rarely seen under the age of 40 years
  4. The symptoms are due to the following:
    1. Hyperviscosity.
    2. Hypervolemia.
    3. Hypermetabolism.
  5. There is a history of epistaxis and bleeding from the GI tract.
  6. There is easy bruising and bleeding.
Polycythemia Rubra Presentation

Polycythemia Rubra Presentation

  1. The patient may have neurologic symptoms like vertigo, tinnitus, headache, and eye problems, particularly after a hot bath, and may become a severe problem.
  2. Hypertension is seen in 1/3 of the patients.
  3. There may be generalized pruritus.
  4. The spleen may be palpable in 75% of the cases (60% to 90%). It is more common in patients with leucocytosis.
  5. Hepatomegaly is less common but seen in 40% to 50% of the cases.
  6. Gout may be seen due to a raised level of uric acid.
  7. Peptic ulceration is seen in 5% to 10% of the patients.
  8. In these patients, there is a tendency to develop peptic ulcer and gout.
  9. There is a tendency to develop venous thrombosis.

How will you diagnose Polycythemia (Rubra) Vera?

  1. Hemoglobin is raised.
  2. Hct may be >60%.
  3. Normal MCV and MCH.
  4. Total red cell mass:
    1. Male >35 mL/Kg
    2. Female = >32 ml/Kg.
  5. Arterial oxygenation is >92%.
  6. There is an increased number of WBCs and platelets. You may see a shift to the left.
    1. A raised platelet count is seen in half of the patients.
  7. Increased leucocytosis is seen in more than half of the patients, and even may see increased basophils.
    1. Increased neutrophil alkaline phosphatase score (NAP).
  8. Bone marrow is hypercellular with prominent megakaryocytes.
  9. The serum erythropoietin level is usually low.
  10. Blood viscosity is increased.
  11. Serum LDH level is normal,
  12. Serum uric acid is increased in up to 40% of the cases due to the turnover of RBCs.
  13. There is splenomegaly.
  14. The JAK2 mutation is seen in granulocytes in almost 100% of the cases.
  15. There is increased serum vitamin B12 and B12-binding capacity.
  16. Typical parameters for the diagnosis are:
    1. Increased RBC mass (Increased RBC and hematocrit).
    2. Leucocytosis and Thrombocytosis.
    3. Splenomegaly.

How will you treat polycythemia Rubra Ver?

  1. Venesection to keep hematocrit low.
  2. Cytotoxic myelosuppression by chemotherapy. Phosphorus-32 therapy is used in older patients.
  3. Interferon (α-interferon) suppresses the bone marrow.
  4. Aspirin prevents thrombotic complications.

What will the prognosis be for polycythemia Rubra Vera?

  • Mostly, the prognosis is good, and the median survival is 10 to 16 years.
  • The major complications are thrombosis and hemorrhage.
  • It may progress to myelofibrosis with myeloid metaplasia in about 20% to 25%.
  • 5% to 6% of the cases end up in acute leukemia.

Secondary polycythemia

  1. Secondary polycythemia is true polycythemia caused by either natural or artificial increases in erythropoietin production, leading to increased RBC production.
  2. RBCs count maybe 6 to 8 million (occasionally 9 million cmm).
  3. Secondary polycythemia resolves when the underlying cause is treated.

What are the causes of Causes of secondary polycythemia?

  • Or physiological polycythemia may be caused by:
  1. High Altitude (altitude sickness).
  2. Hypoxic disease.e.g., cyanotic heart and hypoxic lung disease (COPD). And chronic obstructive sleep apnea.
    1. Smoker’s polycythemia is due to the formation of the carboxyhemoglobin.
  3. Idiopathic or Iatrogenic can be induced directly by phlebotomy, which can concentrate the erythrocytes.
  4. Genetic abnormalities in hemoglobin oxygen release.
  5. Renal diseases like cystic disease and hydronephrosis.
  6. Less common causes are:
    1. Renal-cell carcinoma (1% to 5%).
    2. Liver tumors (Hepatoma = 3% to 12%).
    3. Pheochromocytoma.
    4. Adrenal adenoma with Cushing’s syndrome.
  7. Anabolic steroids use (athletes abuse steroids).
  8. Testosterone replacement for hypogonadism.
  9. People who take erythropoietin may develop secondary polycythemia.

How will you diagnose Secondary polycythemia?

  1. Increased RBC count.
  2. Increased hemoglobin.
  3. Increased hematocrit.
  4. Normal MCV and MCH.
  5. White cells and platelets are normal.

Relative polycythemia (Pseudopolycythemia)

How will you define Relative polycythemia?

  1. A total normal RBC mass falsely appears to have increased due to a decrease in the plasma volume.
  2. Dehydration is the most common cause.

What are the causes of Relative Polycythemia?

  1. It is caused by:
    1. Stress,
    2. Smoking.
    3. Dehydration in case of water deprivation and vomiting.
    4. Plasma loss in burn and enteropathy.

What are the characteristics of Relative Polycythemia?

  1. This is characterized by the following:
    1. Raised hemoglobin.
    2. Normal TLC.
    3. Normal platelets.
    4. Normal red cell mass.
    5. Decreased plasma volume.

What are the laboratory findings of Relative Polycythemia?

  1. Laboratory findings are:
    1. Increased RBC count, Hb, Hct,
    2. Normal WBCs and platelets.
  2. Relative polycythemia is seen in decreased plasma volume and may be seen in dehydration or hemoconcentration.

How will you treat the Polycythemia?

  1. The treatment of the choice is phlebotomy.
  2. Donate one point of blood until the Hct is <45%.
  3. The patient should be given a low-iron diet.
  4. Chemotherapy like myelosuppressive drugs may be given.

How will you summarize various Polycythemias?

Clinical and laboratory findings Relative polycythemia Secondary polycythemia Primary polycythemia Rubra
1. Red cell mass DEcreased/Normal increased Increased
2. Erythropoietin Normal Increased (rarely normal) Decreased (rarely normal)
3. White blood cells(shift to left) Normal Normal Increased in 80% of the cases
4. Platelets count Normal Normal Increased by 50% of the cases
5. Nucleated RBCs Absent Absent Usually present
6. Bone marrow Normal increased erythropoiesis
  1. Hypercellular
  2. Increased erythropoiesis
  3. Leucopoiesis
7. Leucocyte alkaline phosphatase score Normal Normal Increased in 70% of the cases
8. Serum vitamin B12 Normal Normal Increased in 75% of the cases
9. Splenomegaly Absent Absent Present in 75% of the cases
10. Hepatomegaly Absent Absent Present in 35% of the cases
11. Heart or lung disease Absent Present Absent
12. Cyanosis May be present Present Absent

questions and answers:

Question 1: Is there any association of heart/lung disease with polycythemia vera?
Show answer
Mostly heart/lung disease is absent n polycythemia vera.
Question 2: Do you see cyanosis in polycythemia vera?
Show answer
Cyanosis is absent in polycythemia vera.

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