Q. Does polycythemia vera cause high hemoglobin and hematocrit?

Hi,

Welcome to icliniq.com.

I went through your data (attachments removed to protect the patient's identity). Your hemoglobin is at borderline, but your hematocrit is clinically high. Elevated hemoglobin is seen in smokers, people living at high altitudes, congestive cardiac failure, and chronic obstructive pulmonary disease. Some erythropoietin secreting adenomas of kidneys very rarely could be a part of primary polycythemia. Rule out the causes of secondary polycythemia. If all causes are excluded, get JAK2 (janus kinase 2), Exon12, and CALR (calreticulin) mutations tested. Even in primary polycythemia called polycythemia rubra vera, these mutations are negative frequently. In cases of secondary polycythemia, the treatment of elevated hemoglobin is to treat the cause. In primary polycythemia, if JAK2 is negative, the treatment is therapeutic phlebotomy or venesection. The goal is to keep hemoglobin and hematocrit within the range for males, less than 50 percent, and for a female, less than 48 percent. And hemoglobin in males at 16 gm/dL and females up to 15 gm/dL. It can be combined with hydroxyurea which dose could be calculated according to the patient's weight. Repeated venesections or phlebotomies can lead to micronutrient deficiency, especially iron. The patient should be given micronutrient replacement therapy. For JAK2 positive patients, Ruxolitinib Tablets are offered in a dose of 5 to 15 mg per day. Another option is IFN alpha (immune interferon) in low dose (45 ug/week) in both JAK2 positive and negative cases. CALR positive cases show superior results on this mode. It could take years to cure the disease. Meanwhile, along with all therapeutic modalities, thrombophilia prophylaxis is given. This is not a life-threatening disease. The patient can die with this but does not die of it. Stepwise investigations

1) JAK2 mutation test.

2) Echocardiogram.

3) Spirometry.

4) Hemoglobin electrophoresis.

5) Abdominopelvic ultrasound.

I suggest the following treatment :

1) If hemoglobin is less than 16 gm/dL and hematocrit is less than 50 percent with venesection of one to three times a year. No need for drug treatment. If hemoglobin rises briskly (requiring four venesections a year), treatment would depend upon the results of the above-mentioned investigations.

2) As it is a high turnover state of the body, I suggest taking Vitamin B12, Pyridoxine, Folic acid, oral iron, Vitamin D and Vitamin C.

3) Also, take uric acid lowering agents such as Allopurinol 300 mg/day until uric acid is in a normal range.

4) Also, take a blood thinner such as low dose Aspirin 75 mg and Warfarin 5 mg once daily.

Thank you.

Hi,

Welcome back to icliniq.com

In 30 to 40 percent of cases of polycythaemia, JAK 2 is negative. In such cases, it is a diagnosis of exclusion, that is, exclude causes of secondary increase in hemoglobin. In JAK 2 negative cases, we test CALR and EXON 12 mutations. Hope the things are clear.

Thank you.

Same symptoms doesn’t mean you have the same problem. Consult a doctor now!

Dr. Mubashir Razzaq Khan

Experience: 14 + Yrs

Hematology

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