Phlebotomy in Hemochromatosis, Polycythemia vera

  1. Do phlebotomy to keep Transferrin saturation <50% in Hemochromatosis

Iron Overload

Serum Transferrin saturation-Screens

HFE gene testing-Confirms

Bronzed skin

Diabetes

Arthritis

CHF.

Cirrhosis.

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2. Do phlebotomy to keep Ferritin <45% in P Vera?

In Polycythemia vera, due to JAK2 mutation,

Bone marrow has hypercellularity and keeps making WBC, RBC and Platelets.

Erythropoetin and Iron levels go down.

When RBC count increases too much, it can cause Pruritus, Erythromelalgia, Thrombosis which can involve retinal veins too, splenomegaly etc.

We do phlebotomy in the initial stages as cells are too many causing issues.

We give ASA to reduce risk of thrombosis and Hydroxyurea also.

Eventually, marrow burns out leading to Myelofibrosis. At this stage, you can see Tear drop cells, Target cells, nucleated RBCs etc.

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3. JAK 2 mutation is seen in Essential Thrombocytosis also.

In Essential Thrombocytosis,

When platelets count goes up, vaso occlusive symptoms happen. Headaches, livedo reticularis can be seen.

ASA and Hydroxyurea can be used here too.

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4. Hydroxyurea is also used in Sickle cell disease as you already know to increase Hb F.

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5.There is an entity called Livedo Racemosa. In this condition, the lines are Broken. This is an emergency. You have to act fast like in a RACE.

Google a pic and see.

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