Male patient of 52 years old with a two-year history of fatigue and pruritus of his legs , headache . And visual disturbances . He smoked one pack of cigarettes per day as he had for 25 years and had about five to six alcoholic drinks daily. Physical exam was marked hepatosplenomegaly and erythromalagia .

He go to the Doctor …. The Doctor request CBC … the CBC shown very high result of RBCS (Erythrocytosis)

CBC result Normal range result parameter 41-51 63 HCT 14-18 21.9 HB (4.5-6)*10 6 9.96*106 RBC 80-99 90.1 MCV 4.5-10.8 10.1*103 WBC 150-450 154*103 platlete 26-388 9 ferritin 65-175 55 iron 250-450 431 TIBC 22-55 13 Iron saturated 0.2-2.44 1.12 Reticulocyte

EXPLAIN ?

Bone marrow erythroid hyperplasia

THE DIAGNOSE IS …

SECONDARY POLYCYTHEMIA

NLMD HD MM absolute primary PT MF Myeloproliferative PPP secondary apparatus PT MF Lymphoproliferative HD NHL MM

What is polycythemia ?

Polycythemia is a condition that results in an increased level of circulating red blood cells in the bloodstream. People with polycythemia have an increase in hematocrit, hemoglobin, or red blood cell count above the normal limits. Polycythemia is normally reported in terms of increased hematocrit (hematocrit is the ratio of the volume of red blood cells to the total volume of blood) or hemoglobin concentration (hemoglobin is a protein responsible for transporting oxygen in the blood).

Hematocrit (HCT): Polycythemia is considered when the hematocrit is greater than 48% in women and 52% in men. Hemoglobin (HGB): Polycythemia is considered when a hemoglobin level of greater than 16.5g/dL in women or hemoglobin level greater than18.5 g/dL in men.

Polycythemia can be divided into two categories; Absolute and apparents: *ABSOLUTE POLYCYTHEMIA : 1. Primary polycythemia: In primary polycythemia the increase in red blood cells is due to inherent problems in the process of red blood cell production. 2. Secondary polycythemia: Secondary polycythemia generally occurs as a response to other factors or underlying conditions that promote red blood cell production.

*APPARENT POLYCYTHEMIA: is defined as those cases where an increased venous PCV is not explained by an increased red cell mass (RCM) but by a reduction in the plasma volume (PV), or by the combination of an RCM towards the upper limit of normality and a PV towards the lower limit of normality.

Red cell production (erythropoiesis) takes place in the bone marrow through a complex sequence of tightly regulated steps. The main regulator of the red cell production is the hormone erythropoietin (EPO). This hormone is largely secreted by the kidneys, although, about 10% may be produced and secreted by the liver. Erythropoietin secretion is up-regulated in response to low oxygen levels (hypoxia) in the blood. More oxygen can be carried to tissues when erythropoietin stimulates red blood cell production in the bone marrow to compensate for the hypoxia.

What are the causes of primary polycythemia? Primary polycythemias are due to acquired or inherited genetic mutations causing abnormally high levels of red blood cell precursors. Primary familial and congenital polycythemia (PFCP) and polycythemia vera (PV) are in this category. Polycythemia vera Polycythemia vera is typically associated with an elevated white blood cell count (leukocytosis) and platelet count (thrombocytosis). An enlarged spleen (splenomegaly) and low erythropoietin levels are other clinical features of polycythemia vera.

genetic mutations of the JAK2 gene were found to be responsible for most cases of polycythemia vera. These mutations are thought to possibly increase the sensitivity of the red blood cell precursors to erythropoietin, thereby, increasing red blood cell production.

What are the common causes of secondary polycythemia? Secondary polycythemia is usually due to increased erythropoietin (EPO) production either in response to chronic hypoxia (low blood oxygen level) or from an erythropoietin secreting tumor. A rare genetic condition, called Chuvash polycythemia, causes increased activity of the gene that produces erythropoietin. The overproduction of erythropoietin causes polycythemia.

What is relative polycythemia? In some forms of secondary polycythemia, the hemoglobin or red blood cell count is perceived to be abnormally high due to an increased concentration of blood. This can happen as a result of plasma volume loss from dehydration, severe vomiting or diarrhea, or excessive sweating. Polycythemia in these situations may be called relative polycythemia, because the actual numbers of red blood cells are not abnormal

weakness fatigue headache itching bruising joint pain dizziness Symptoms of polycythemia include: weakness fatigue headache itching bruising joint pain dizziness abdominal pain

What causes the itching What causes the itching? Most likely it is from the release of histamines from the BASOPHILE caused by various stimuli such as air and hot or cold water.

Treatment The main aim is to reduce thrombotic complication. " Debulking" by removal of RBCs and platelet by apheresis or by repeated phlebotomy. The median survival may be as long as 15 years. Additional treatment may not be needed and is controversial(Chemotherapy).

THE END