Pathophysiology - Lecture 11 - Blood & Circulatory Disorders - Polycythemia

Polycythemia

excess of red blood cells

s/s polycythemia

-distended blood vessels, sluggish blood flow

-increased BP

-Hypertrophied heart

-Hepatomegaly

-Splenomegaly

-Dyspnea

-Headaches

-Visual disturbance

-Thromboses and infarctions

Why do polycythemia pt blood pressures increase?

blood is like syrup because of too many RBCs

Dx test for polycythemia

-increased cell counts

-increased hemoglobin and hematocrit values

-hypercellular bone marrow

-hyperuricemia

Hyperuricemia

high uric acid

Tx for polycythemia

-suppression of bone marrow

-periodic phlebotomy

Why periodic phlebotomy in polycythemia patients?

to reduce amount of blood in body

Indications of blood-clotting disorders

-persistent bleeding from gums

-repeated epistaxis

-petechiae

-frequent purpura and ecchymosis

-more than normal bleeding in trauma

-bleeding into joint

-hemoptysis

-hematemesis

-blood in feces

-anemia

-feeling faint and anxious

-low BP

-Rapid pulse

epistaxis

nose bleeding

Petechiae

pinpoint, flat, red spots on skin and mucous membrane

ecchymosis

bruising

Hemoptysis

coughing up bloody sputum

Hematemesis

coarse brown particles (coffee ground emesis)

Blood in feces color and indicates what?

black and occult

Hemophilia A

Deficit or abnormality of factor VIII

Causes of Hemophilia A

-genetic

-X-linked recessive

-manifested in men, carried by women

s/s hemophilia A

-prolonged bleeding after minor tissue trauma

-spontaneous bleeding into joints

-possible hematuria

-possible blood in feces

-varying degrees of severity

Dx test of hemophilia A

-PTT

-bleeding time and PT normal

-coagulation time prolonged

-Serum levels of factor VIII are low

tx for Hemophilia A

-Desmopressin (DDAVP)

-Advate

Advate

-replacement therapy for factor VII

-Artificial factor VIII

Von Willebrand's Disease

-most common hereditary clotting disorder

-men and women

-factor VIII jeopardized in 3 major ways

s/s Von Willebrand's Disease

-skin rashes

-frequent nosebleeds

-easy bruising

-bleeding of gums

-abnormal menstrual bleeding

tx for Von Willebrand's Disease

based on type and severity

What is Disseminated Intravascular Coagulation (DIC)?

A condition that involves both excessive bleeding and clotting.

What occurs during Disseminated Intravascular Coagulation?

Excessive clotting in circulation.

What happens to clotting factors in Disseminated Intravascular Coagulation?

Clotting factors are reduced to a dangerous level.

What is a consequence of Disseminated Intravascular Coagulation?

Widespread, uncontrollable hemorrhage results.

Prognosis of DIC

very poor prognosis, with high fatality fate 50-70%

Complication of many primary problems such as

-pregnancy (specifically during birth)

-Infections

-carcinomas

-Major Trauma

Tx for DIC

-treat underlying cause

-increase platelet production

Thrombophilia

Abnormal clots in veins or arteries

What is Hereditary Hemochromatosis?

A genetic disorder characterized by iron overload.

What happens to iron in Hereditary Hemochromatosis?

Iron accumulates in a number of body tissues.

At what age do symptoms of Hereditary Hemochromatosis typically develop?

In persons older than 40.

What is a consequence of absorbing too much iron in Hereditary Hemochromatosis?

It damages the organs.

What visceral permits excessive iron absorption?

intestinal lining

What may help slow down HH?

low iron diet

What organs would be damaged the most if HH goes untreated?

-liver

-heart

3 phases of HH

-phase of latency

-phase of biochemical expression

-phase of clinical expression, symptomatic

Why do symptoms of HH take decades?

because of the build up iron

Dx of HH

-Check blood for iron

-genetic testing

-iron accumulated in liver

Tx for HH

periodic phlebotomy

Untreated Prognosis of HH

-Cardiac failure

-liver failure

-liver cancer

Treated Prognosis of HH

-found early

-properly managed

-longer life expectancy

-not differ from that of persons without the disorder

Myelodysplastic Syndromes

disease that involve inadequate production of cells by the bone marrow

s/s myelodysplastic syndrome

-anemia

-dependent on type of deficiencies that occur

-bone marrow is compromised

-exposure to chemo?

-exposure to chemical?

tx to myelodysplastic syndromes

-transfusion replacement

-chelation therapy to reduce iron overload

-bone marrow transplantation

Leukemias

Group of neoplastic disorders involving white blood cells

What happens to WBCs in leukemia?

uncontrolled WBC production in bone or lymph nodes

Leukocytes growth in Leukemia

-undifferentiated

-immature

-nonfunctional

4 Leukemias

-Acute Lymphocytic Leukemia (ALL)

-Acute Myeloid Leukemia (AML)

-Chronic Lymphocytic Leukemia (CLL)

-Chronic Myeloid Leukemia (CML)

ALL and AML

-high proportion of immature nonfunctional cell in bone marrow and peripheral circulation

-CHILDREN and younder adults

CLL and CML

-higher proportion of mature cells

-common in older adults

s/s of Acute Leukemia

-frequent/uncontrolled infections

-petechiae adn purpura (bleeding)

-signs of anemia

-severe and steady bone pain

-weight loss

-fatigue

-possible fever

-enlarged lymph nodes, spleen, liver

-headache

-visual disturbances

-drowsiness

-vomiting

Dx test of Leukemia

-Peripheral blood smears

-immature leukocytes and altered numbers of WBCs

-Numbers of RBCs and platelets decreased

-Bone marrow biopsy for confirmation

Tx for Leukemia

-chemotherapy

-biological therapy

-Radiation

-bone marrow transplant

-ALL in young children responds well to Chemo

-Interferon

complications of leukemia

-opportunistic infections, including pneumonia

-sepsis

-CHF

-Hemorrhage

-Liver failure

-Renal failure

-CNS depression and coma

Multiple Myeloma

Neoplastic disease that involves increased production of plasma cells in bone marrow

Pathway of Multiple Myeloma

B cells to Plasma cells

Plasma cells to cancerous (plasmacytoma)

multiple tumors in bone become multiple myeloma

affect MM has on bones

-loss of bone

-severe bone pain

-skull and vertebrae crumble like egg shells

Prognosis of MM

prognosis poor, with short life expectancy

What is the typical onset of multiple myeloma?

Onset usually insidious

How advanced is multiple myeloma at the time of diagnosis?

Malignancy well advanced before diagnosis

What causes pain in multiple myeloma?

Pain caused by bone involvement

What blood condition is associated with multiple myeloma?

Anemia and bleeding tendency

What is a common kidney issue in multiple myeloma?

Impaired kidney function and eventually failure

What treatment is used to encourage remission in multiple myeloma?

Chemotherapy