5Th Quarter Hematology Part 5

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Condition characterized by abnormal increase in production of red blood cells

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Polycythemia

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Characterized by hematocrit greater than 50%

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Polycythemia

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Overproduction of red blood cells, white blood cells and platelets

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Polycythemia vera, primary polycythemia

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Proliferative disorder of the myeloid stem cells, the bone marrow is hyper Cellular and the erythrocyte, leukocyte and platelet counts in the peripheral blood are often elevated

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Primary polycythemia, polycythemia vera

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Hematocrit can exceed 60%

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Polycythemia Vera, primary polycythemia

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This is a acquired disorder of bone marrow occurring in more men than women and usually at over 50 years of age

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Polycythemia vera

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Too many blood cells produced by the marrow leads to thickening of blood and thus congestion of organs

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Polycythemia vera

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With treatment the median survival exceeds 10 years. Death typically results from thrombosis, Hemorrhage or more rarely Evolution to AML

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Polycythemia vera

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Ruddy complexion and splenomegaly

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Polycythemia vera

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Symptoms result from increased blood volume and may include headache, dizziness, tinnitus, fatigue, paresthesias and blurred vision

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Polycythemia vera

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Symptoms can result from increased blood viscosity and can include angina, claudication, thrombophlebitis

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Polycythemia vera

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Splenomegaly and hepatomegaly

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Polycythemia vera clinical manifestations

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Uric acid may be elevated resulting in gout and renal stones

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Polycythemia vera

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Generalized pruritus may be caused by histamine release and increased number of basophils

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Polycythemia Vera

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Erythromelagia which is a burning sensation in the fingers and toes may be reported

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Polycythemia vera

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Clinical manifestations of this condition have to do with too much blood volume and increased viscosity

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Polycythemia vera

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In this type of polycythemia the occurrence is due to Chronic hypoxia which stimulates increased production of red blood cells

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Secondary polycythemia

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Risk factors for this type of polycythemia are heavy smoking / COPD / high altitude

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Secondary polycythemia

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Diagnosis is based on an elevated erythrocyte Mass, normal oxygen saturation level and often and enlarged spleen

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Polycythemia

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Patients with this condition are at increased risk for thrombosis that may result in Strokes or heart attacks. Thrombotic complications are the most common cause of death

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Polycythemia

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Bleeding is another complication of this disorder possibly due to platelets being large and somewhat dysfunctional

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Polycythemia

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Objective of management in this condition is to reduce the high RBC count and reduce the risk of thrombosis

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Polycythemia

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Phlebotomy is an important part of therapy for this condition. It involves removing enough blood to reduce blood viscosity and to deplete the patient's iron stores thereby rendering the patient iron deficient and unable to continue manufacturing excessive hemoglobin

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Polycythemia

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Many patients with this condition are managed by routine phlebotomy on an intermittent basis with the target of maintaining the hematocrit less than this percentage

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Polycythemia

HCT less than 45%

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During phlebotomy treatments for polycythemia approximately how much blood is removed and how often

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Initially 500ml once or twice weekly

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Chemotherapeutic agents can be used to suppress marrow function thereby controlling blood counts in this condition

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Polycythemia

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This medication which inhibits platelet aggregation has been used to control thrombocytosis associated with this condition

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Anagrelide

Polycythemia

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List two medication classifications used in treating polycythemia

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Myelosuppressive, inhibit marrow activity

Anti platelets to prevent thrombosis

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Interferon Alpha 2B, is effective in controlling blood counts in this disorder. However it may be hard to tolerate do to side effects and cost.

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Polycythemia

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Pruritus is very common occurring in 70% of these patients. It is one of the most distressing symptoms of the disease. Antihistamines including H2 blockers are not effective in controlling itching. Interferon alfa-2b is the most effective treatment

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Polycythemia

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Chemotherapeutic agents such as hydroxyurea can be used to suppress marrow function and control blood counts in these patients

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Polycythemia

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High-dose aspirin may be associated with an increase in risk of bleeding and no decrease in risk of thrombosis. Whereas low dose aspirin decreases thrombotic complications but does not increase bleeding therefore it is recommended

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Polycythemia

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Hydration is very important in this condition to decrease viscosity however one must be careful not to overload because they already are experiencing an excess

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Polycythemia

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Risk factors for thrombotic complications of this condition include smoking, obesity, poorly controlled hypertension and diabetes

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Polycythemia

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To reduce likelihood of DVT in this condition sedentary Behavior, Crossing legs, wearing tight or restricting clothing should be discouraged

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Polycythemia

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Minimize alcohol intake to decrease risk of bleeding. Instruct patient to avoid iron supplements including over-the-counter multivitamins because the iron can further stimulate red blood cell production

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Polycythemia

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Neoplasms of cells of lymphoid origin

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Lymphoma

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Tumors usually start in lymph nodes that can involve lymphoid tissue in the spleen, GI tract, liver or bone marrow

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Lymphoma

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Lymphomas can be broadly classified into two categories which include

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Hodgkin's lymphoma and Non-Hodgkin's lymphoma

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Bone marrow or lymph is where this begins

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Lymphoma

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Relatively rare malignancy with a high cure rate

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Hodgkin lymphoma

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Somewhat more common in men, with two peaks of incidence. One occurring at 15 to 35 years of age and the other occurring at greater than 50 years of age

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Hodgkin lymphoma

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Although cause is unknown it may have a familial pattern. There is a possible link to Epstein-Barr virus and exposure to toxins such as Agent Orange

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Hodgkin's lymphoma

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Unlike other lymphomas this variety originates in a single node. The disease spreads by contiguous extension along the lymphatic system

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Hodgkin lymphoma

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The malignant sell of Hodgkin lymphoma is this

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Reed Sternberg cell which are thought to be of lymphoid origin

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the cause of this condition is unknown but a viral etiology is suspected. Including things like Epstein-Barr virus, HIV and herpes

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Hodgkin lymphoma

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Usually begins as an enlargement of one or more lymph nodes on one side of the neck. The nodes are painless and firm but not hard

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Hodgkin lymphoma

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The most common sites for lymphadenopathy are the cervical, supraclavicular and mediastinal nodes

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Hodgkin lymphoma

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All organs are vulnerable to Invasion by tumor cells. Symptoms result from compression of organs by the tumor such as a cough and Pulmonary effusion, jaundice or abdominal pain.

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Hodgkin lymphoma

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Enlarged non tender lymph nodes, fatigue, night sweats, weight loss so, fever

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Hodgkin's lymphoma

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Can spread to other organs such as the lungs, spleen and liver

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Hodgkin's lymphoma

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Additional symptoms vary depending on tumor location such as an abdominal Mass interfering with bowels, or a liver Mass leading to jaundice

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Hodgkin's lymphoma

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Mild anemia is the most common hematologic finding of this condition.

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Hodgkin's lymphoma

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Patients with this condition have impaired cellular immunity as evidenced by an absent or decrease reaction to skin sensity tests

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Hodgkin's lymphoma

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Diagnosis is made by means of an excisional lymph node biopsy and the finding of reed-sternberg cells

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Hodgkin's lymphoma

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A cluster of symptoms has important prognostic implications. Referred to as b symptoms they include fever without chills, drenching sweats especially at night, unintentional weight loss of more than 10%

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Hodgkin's lymphoma

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Assess for any b symptoms, palpable lymph node chains and size of spleen and liver

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Hodgkin lymphoma

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The goal in the treatment of Hodgkin lymphoma is

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Cure

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Treatment for this is determined primarily by the stage of the disease not the histologic type

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Hodgkin's lymphoma

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Treatment of limited stage Hodgkin lymphoma commonly involves

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Short course of chemo followed by radiation

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Combination chemotherapy with doxorubicin, bleomycin, vinblastine, and dacarbazine is considered standard treatment for more advanced disease

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Hodgkin's lymphoma

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Chemotherapy is often successful in obtaining remission even when relapse occurs. Transplant is used for advanced or refractory disease

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Hodgkin's lymphoma

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Most patients diagnosed with this condition are either cured or experienced prolonged remissions and live many years post diagnosis

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Hodgkin's lymphoma

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Teach that secondary malignancy is possible after cure of this condition. So avoid risk factors such as tobacco and alcohol exposure

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Hodgkin's lymphoma

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This type of lymphoma occurs more commonly

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Non-Hodgkin's

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Neoplasms of the lymphoid system.

Most often involving malignant B lymphocytes.

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Non-hodgkin lymphoma

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In contrast to Hodgkin lymphoma, the lymphoid tissues involved are largely infiltrated with malignant cells. The spread of these malignant unemployed cells occurs unpredictably and true localized disease is very uncommon

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Non-Hodgkin's lymphoma

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Lymph nodes from multiple sites maybe infiltrated as May sites outside the lymphoid system. Localization is uncommon, metastasis is more common

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Non-Hodgkin's lymphoma

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7th most common type of cancer in the US. The incidence increases with each decade of Life the median age of diagnosis is 65 years

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Non-Hodgkin's lymphoma

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One third of patients with Non-Hodgkin's lymphoma have b symptoms

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True

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Lymphomatous masses can compromise Organ function just as with Hodgkin's lymphoma

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Non-Hodgkin's lymphoma

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Painless lymph node enlargement, fever, weight loss, night sweats

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Non-Hodgkin's lymphoma

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Usually already spread by the time of diagnosis

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Non-Hodgkin's lymphoma

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Treatment for these conditions is based on staging

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Hodgkin's and Non-Hodgkin's lymphoma

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This lymphoma is more difficult to diagnose and treat, and the prognosis is not as good

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Non-Hodgkin's lymphoma

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The most commonly used treatment methods for lymphomas are chemotherapy and radiation

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True

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Chemotherapy causes systemic side effects whereas radiation therapy cause a specific side effects limited to the area being irradiated

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True

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Many lymphomas can be cured with current treatments. However as survival rates increase the incidence of second malignancies particularly AML or MDS also increases. So survivors should be screened regularly for the development of second malignancies

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True

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Treatment is usually in outpatient settings unless complications arise

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Lymphoma

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4 teaching areas for lymphoma

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Chemo and radiation

Risk for infection

Nutrition

Teaching about tumor location

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The video for Babs the crab represents what condition

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Multiple myeloma

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Cancer of the plasma cells

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multiple myeloma

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Malignant plasma cells produce too much immunoglobulin

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Multiple myeloma

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Malignant disease of the most mature form of B lymphocyte, the plasma cell

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Multiple myeloma

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Plasma cells secrete immunoglobulins which are proteins necessary for

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Antibody production to fight infection

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Second most common hematologic cancer in the US

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Multiple myeloma

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The 5 year median survival rate for newly diagnosed patients of this condition is 39%

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Multiple myeloma

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Currently prognosis of this is based on two markers including serum albumin and serum beta 2 microglobulin

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Multiple myeloma

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Malignant plasma cells produce increased amounts of immunoglobulin that is non-functional

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Multiple myeloma

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Functional types of immunoglobulin are still produced by non-malignant plasma cells although in lower than normal quantities.

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Multiple myeloma

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The specific immunoglobulins secreted by the myeloma cells is detectable in the blood or urine and is referred to as mononucleosis protein or M protein. This protein serves as a useful marker to monitor the extent of disease in the patient's response to therapy

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Multiple myeloma

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Malignant plasma cells also secretes certain substances to stimulate the creation of new blood vessels called angiogenesis. This enhances the growth of clusters of plasma cells

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Multiple myeloma

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The classic presenting symptom of this condition is bone pain usually in the back or ribs.

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Multiple myeloma

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The bone pain associated with this condition increases with movement and decreases with rest, patients May report that they have less pain on awakening but more during the day

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Multiple myeloma

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A substance secreted by plasma cells, osteoclast activating Factor is involved in stimulating osteoclasts. This causes bone break down. Thus lytic lesions and osteoporosis are often seen.

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Multiple myeloma

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Bone destruction can be severe enough to cause vertebral collapse, fractures and spinal cord compression

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Multiple myeloma

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Bone destruction can lead to excessive calcium loss and entering the serum leading to hypercalcemia. Renal failure may also then occur.

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Multiple myeloma

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As more and more malignant plasma cells are produced marrow has less space for RBC production and anemia May develop. Late in the disease A reduced number of leukocytes and platelets may also be seen due to bone marrow infiltration by malignant plasma cells

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Multiple myeloma

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The diagnosis of myeloma is confirmed by bone marrow biopsy. The presence of More Than This percentage of plasma cells is the homework diagnostic criteria

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More than 10% plasma cells more than 10% plasma cells

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Radiation therapy is very useful in strengthening the bone at a specific Lesion, particularly one at risk for bone fracture or spinal cord compression. It is also useful in relieving bone pain and reducing the size of plasma cell tumors that occur outside the skeleton.

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Multiple myeloma

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The diagnosis of myeloma is confirmed by bone marrow biopsy. The presence of More Than This percentage of plasma cells is the hallmark diagnostic criteria

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More than 10% plasma cells

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Occurs most commonly in older people and infection is often the cause of death

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Multiple myeloma

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Hollow needle position within the fractured vertebrae and when the precise location is confirmed an Orthopedic cement is infiltrated into the vertebrae to stabilize a fracture and strengthen it. For most patients relief from the pain is almost immediate

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Vertebroplasty for multiple myeloma

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Because the infiltration of the marrow by these malignant plasma cells is not uniform the plasma cells may not be increased in a given sample, a false negative result

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Multiple myeloma

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Anemia, fractures, renal impairment, infections, neuropathy

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Other symptoms of multiple myeloma

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There is no cure for multiple myeloma

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True

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For many patients it is possible to control the illness and maintain level of functioning quite well for several years or longer. For those who are not candidates for hsct, chemotherapy is the primary treatment

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Multiple myeloma

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Chemotherapy may be used in combination with corticosteroids such as dexamethasone (decadron) in order to increase the effectiveness of the chemo

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Multiple myeloma

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Surgery is a possible treatment for this condition in order to help stabilize the spine due to bone break down

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Vertebroplasty for multiple myeloma

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Pain management is very important in patients with multiple myeloma. NSAIDs can be very useful for mild pain or can be administered in combination with opioid analgesics. Because NSAIDs can cause gastritis and renal dysfunction, renal function must be carefully monitored and patience assessed for gastritis

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Multiple myeloma

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Educate patient about activity restrictions such as not lifting more than 10 lb. Braces are occasionally needed to support the spinal column

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Multiple myeloma

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Premature separation of the placenta from the uterine wall

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abruptio placentae

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Mild to severe abdominal pain and abdominal rigidity

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Abruptio placentae

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Abruptio placentae usually occurs during this trimester and effects this many

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Third trimester, one in 1,000 births

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Treatment depends on severity of blood loss and Fetal maturity

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Abruptio placentae

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75% of these cases will have vaginal bleeding

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Abruptio placentae

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Excessive blood loss less than 24 hours after childbirth

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Early postpartum hemorrhage

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Excessive blood loss up to 6 weeks after childbirth

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Late postpartum hemorrhage

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Overdistention of uterus, uterine fatigue, retained placenta, pre-eclampsia, induced labor, prolonged labor, multiparity

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Causes of postpartum hemorrhage

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Most frequent cause of postpartum hemorrhage

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Uterine atony

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Complication of postpartum hemorrhage

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Hypovolemic shock

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Fundal massage, notify MD, assess for signs and symptoms of shock, oxytocics, oxygen

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Initial treatment of postpartum hemorrhage