Flashcards in UA WI18-Chapter 10 Blood Disorders Part 1 Deck (131)
A decrease in the number of RBC or less than normal quantity of Hb in blood.
Increased amount of RBC in blood.
What are the three main classes of anemia?
Excessive blood loss
Deficient RBC production
Excessive blood cell destruction
Sx. Common to all anemia.
Weakness, fatigue, pallor, malaise, dyspnea, tachycardia, murmur(systolic), low blood pressure, dizziness, headaches coldness, weak pulse etc.
Acute blood loss is caused _______ and results in _______.
Trauma, surgery, GI tract lesions, Gynecolgic disturbance
Results in hypovolemia
Term for decreased blood volume
In an acute blood loss case report, what would be found?
Increased neutrophils (shift left)
Changes in RBC morphology: normal initially—> hypochromic once volume restored
Chronic blood loss is caused by______.
When do symptoms appears?
Cancer, menstruation, fibroids in older women and angiodysplasia.
When iron store is depleted
In a chronic bleeding case report, what is reported?
RBC morphology: Normocytic or microcytic/ hypochromic
What are the causes of deficient RBC production?
Vit. Deficiency anemia
Bone marrow or stem cell problem
Health disorders: i.e renal insufficiency
Iron deficient anemia is described as?
Low RBC amount or low Hb
Iron deficiency anemia causes?
Iron loss from bleeding
Increased need- e.g growth spurt
Iron deficiency clinical presentation?
Koilonychia-spoon shaped brittle nails
Iron deficiency hematology report?
Megaloblastic anemia is described as?
A condition is which the bone marrow produces unusually large, structurally abnormal immature RBC
Causes of megaloblastic anemia
Vit. B12 , Vit. B9 or both
(Both needed for maturation of blood cell and normal nerve cell function)
Abnormal DNA synthesis and Delayed cell division, with normal Hb & RNA synthesis in megaloblastic anemia is a result of ?
B9 and B12 deficiency
Etiological factors of B12 deficiency
Lack of IF
Increased used (pregnancy)
Etiological factors of B9 deficiency
Malabsorption- duodenum, jejunum
Increased use (pregnancy)
Clinical manifestation of megaloblastic anemia a/w B12 deficiency?
Fatigue, dyspnea, muscle weakness, glossitis, GI problems
Megaloblastic anemia a/w with B12 deficiency will result in _______ which is characterized by_______.
Result in: irreversible nervous system pathology collectively know as Subacute combined degeneration of spinal.
Characterized by: Demyelination of posterior and lateral columns and psychological problems.
What is the AKA term for subacute combined degeneration of spinal cord?
Lichtheim disease is clinically manifested by:
Numbness/tingling in fingers extremities
Loss of balance, vibration and ataxia
Tx for megaloblastic anemia a/w B12 deficiency
B12 by supplement
Injection of b12
Autoimmune disorder in which the body can’t make enough healthy RBC because it can’t absorb sufficient amounts og B12.
Pernicious anemia produces Ab against_____?
Intrinsic factor receptors in ileum
Congenital pernicious is described as
Pernicious anemia passed down.
-Babies w/ this can’t make enough IF or absorb B12 in small intestines so tx requires intramuscular B12 shots-
Megaloblastic anemia a/w B9 clinical manifestation
Angular cheilitis/glossitis, fatigue, dyspnea, muscle weakness, GI problems and Depression.
No Neurological symptoms
Megaloblastic anemia a/w B9 affects pregnancy in what way?
Neural tube defects
Low birth rates
Megaloblastic anemia hematology
Hypersegmented neutrophils (senile)
Morphology: Macrocytic, anisocytosis/poikilocytosis- Howell-jolly bodies
MCV/ MCH increase or normal
Disease in which the bone marrow and the blood stem cells are damaged.
Causes of aplastic anemia
Infection: Ds- hepatitis B/C, Epstein-Barr, Ss-parvovirus
AI (e.g SLE)
Aplastic anemia clinical manifestations
Fatigue, pallor, tachycardia/palpitations/dyspnea, weakness
Thrombocytopenia results from aplastic anemia, what are associated risk?
Leukopenia in aplastic anemia is associated with what risk?
Risk of Infection
Definition: low counts of immature RBC/reticulocytes
(Result of Aplastic anemia)
Aplastic anemia laboratory findings
Decreased granulocytes (WBC)
Increased serum ion (no RBC to take them)
Anemia in which the bone marrow and liver produce ringed sideroblast rather than healthy red blood cells.
Define a sideroblast
Atypical abnormal uncleared erythoblast w/ iron granules
Why do sideroblast form ?
Due to reduce porphyria synthesis which helps incorporate iron into heme
Sideroblastic anemia etiology
30% acquired (MM, SLE, Liver cirrhosis etc)
20% hereditary (X-linked)
Sideroblastic anemia Sx/signs
Enlarged liver and spleen (MC)
Sideroblastic anemia lab finding
Ring sideroblast in bone marrow
Anisocytosis/poikilocytosis/ basophillic stippling, target cells
Increased serum iron & transferrin saturation
Anemia due to hemolysis, the abnormal breakdown of RBC
(Intravascular or extravascular)
Intrinsic etiology of hemolytic anemia include
Defect of RBC membrane production
Defect in hemoglobin production
Defective RBC metabolism
Extrinsic etiology of hemolytic anemia include
Drug side effects
Sx of hemolytic anemia
Shortness of breath
Gallstone (due to increased bilirubin)
Pulmonary hypertension (due to abnormal bilirubin)
Hemolytic anemia lab findings
Increased serum bilirubin (feces and urinary as well)
Intrinsic hemolytic anemia characterized by the production of RBCs that are sphere-shaped and prematurely breakdown.
Hereditary spherocytic anemia
The sphere shaped RBC a/w hereditary spherocytic anemia is a result of ?
Membrane lipoprotein defects
Sx of hereditary spherocytosis
Aplastic crisis (temporary)
Hereditary spherocytosis lab
RBC- microcytic, hyperchromic
MCV low/ MCH normal
Increased unconjugated bilirubin in blood
Increased urobilinogen in ruins and stool
Intrinsic anemia disorder a/w an inherited autosomal recessive hemoglobinopathy, characterized by structurally abnormal Hb.
Sickle cell (group of disorders)
Sickle cell occurs when the _____, ______ on the ______ in the Hb molecule is replaced by other amino acids
Sixth amino acid
The three detected mutation of sickle cells are
HbS- glutamic replaced with valine (E6V)
HbC- glutamic replaced with lysine (E6K)
HbE- Glutamic replaced with lysine on the 26 position on beta globin (E26K)
Sickle cell disease found in which geographic locations?
African, Caribbean, Mediterranean, middle eastern, East Indian ancestries
Sickle cell type that is the most common and occurs when copies of Hb S gene are inherited from both parents.
Hb SS sickle cell anemia AKA E6V
Sickle cell type that is the second most common and occurs when the Hb C gene is inherited from one parent and the Hb S from another
E6K AKA Hb SC sickle cell
Sickle cell type that occurs when the Hb S gene is inherited from one parent and the Hb E from another.
Hb SE AKA E26K
Sickle cell type that occurs when hemoglobin C is inherited from both parents.
Hb CC sickle cell AKA E6K
Sickle cell trait (SCT) is characterized by
Inheritance of Hb S and a normal gene Hb A- don’t have symptoms but can pass it on
Clinical manifestation of Sickle cell characterized by severe anemia.
(Not present younger than 4-6months)
Due to hemolysis-Fatigue, tachycardia, jaundice, dyspnea, paleness
Clinical manifestation of sickle cell characterized by vasooclusion:
(Not present younger than 4-6months)
Painful crisis & Tissue ischemia
low back pain, joint pain
Hand-foot syndrome/ SC dactylitis
Sickle Lab findings
Increased sickle cell Hb, decreased normal
Howell jolly bodies
High reticulocyte count
Blood disorder that is autosomal recessive characterized by abnormal forms of hemoglobin resulting in weakening and destruction of RBC.
Two types of thalassemia based on part of O2 carrying protein RBC is lacking
Beta- less severe
Both alpha and beta forms of thalassemia include the following form.
Major (from both parents)
Minor ( one parent)
In which form of thalassemia are children normal at birth, then exhibit severe anemia.
Also it is more prevalent in Mediterranean people known as Cooley’s anemia
In which form of thalassemia can unborn babies die
The alpha and beta thalassemia minor are characterized by:
Beta-thalassemia major is manifested by:
Increased risk of infection
Bone deformities a/w thalassemia
Hair on end skull- accentuated trabeculae
Honeycomb pattern- destruction due to multiple bone infarcts
Thalassemia lab findings:
Decreased Hb, RBC, MCV, MCH, MCHC
Hypochromic microcytic, poikilocytosis, target cells, Cabot rings, ovalocytosis, Howell jolly bodies
Increased bilirubin, iron, ferritin
Tx of thalassemia
Major forms-regular blood transfusion, folate supplements, bone marrow transplant, chelation therapy (reduce iron)
X-linked recessive genetic disorder that predisposes to spontaneous destruction of RBC and resultant jaundice in response to triggers like:
Illness, food, medication
G6PDH deficiency AKA Favism
Maintains pathway that produces gluthione, an antioxidant
G6PDH deficiency clinical manifestion
Shortness of breath
G6PDH deficiency lab
Bite RBC (Degmacytes)
Heinz bodies w/ inclusions
WBC increase/platelet unaffected
Increased indirect bilirubin, LDH
Extrinsic hemolytic anemias etiological factors
Paroxysmal nocturnal hemoglobinuria
Erythroblastosis fetalis is described as
An occurrence when an Rh(-) mother gives birth to Rh(+) .
Her body synthesis Ab against Rh(+), next Rh(+) is not conceived
State in which the proportion of blood volume that is occupied by red blood cells increases
Two forms of polycythemia
Absolute- absolute #RBC increased
Relative- decrease in volume of plasma w/ normal absolute RBC
What is the hallmark of polycythemia?
Elevated hematocrit w/ normal MCV
Polycythemia present when excessive amount of red blood cells is produced as a
result of an abnormality of the bone marrow.
Absolute primary polycythemia AKA polycythemia Vera
Sx of polycythemia Vera
Itching/redness of skin
Sudden severe burning pain in hands and feet
Headache and vertigo
Polycythemia caused by either natural or artificial increases in the production of erythropoietin.
Characterized only by increased production of RBC
Absolute secondary polycythemia/ Physiological polycythemia
Two conditions associated with absolute secondary polycythemia
1. Conditions that result in physiological appropriate polycythemia (adaptation)
2. Conditions that don’t result as a manifestation of physiological adaptation
Relative polycythemia results from
Loss of body fluid - burns, dehydration, stress.
Type of cancer of blood, bone marrow and lymphoid system characterized by an abnormal increase in immature or mature abnormal white blood cells
Leukemia is a broad base of diseases also know as?
Classification of leukemia characterized by a rapid increase in the number of immature blood cells due to bone marrow disorder
Acute (MC in young/middle aged people)
Classification of leukemia characterized by the excessive building up of RELATIVELY mature and still abnormal WBC
Chronic leukemia (MC in older people)
Leukemia classified based on Lymphocytes being affected
Lymphocytic / lymphoblastic leukemia’s
Leukemia classified based on RBC, some WBC and platelets being affected.
What are the general clinical manifestations of acute forms of leukemia
Flu-like symptoms:fatigue, fever, malaise
Acute lymphocytic leukemia described as?
Malignant immature lymphocytes growing w/in bone marrow.
Affects young children
Blood shows undifferential immature lymphocytes- (blueberries)
Acute myeloid leukemia is described as ?
Rapid growth of abnormal non-differentiated granulocytes, abnormal myeloblasts (WBC) accumulating in bone marrow causing a drop in RBC, Platelets and normal WBC amount.
More often adults, avg age 67 year/ MC in males
Clinical manifestation of chronic leukemia’s
Non specific Sx
Bacterial, viral, fungal infection due to immunosuppression
Chronic lymphocytic leukemia is described as?
Abnormal growth of B cells in the bone marrow that most often affects middle-age adults or elder over the age of 55 and never children
Most people Dx with Chronic lymphocytic leukemia are diagnosed without sx as a result of what
As a result of routine blood test that returns high in WBC count
Form of leukemia classified by the increased and unregulated growth of predominantly myeloid cells (granulocytes) in bone marrow and the accumulation of these cells in the blood, lymphnodes and spleen
Chronic myeloid leukemia
Most people dx with CML have a genetic abnormality in their blood cells called Philadelphia (Ph) chromosome.
Describe the Ph chromosome
The Ph chromosome cause the production of the enzyme tyrosine kinase protein that is always on causing the cell to divide uncontrollably
What is the affect of CML on cells?
Increased granulocytes of all types including mature myeloid cells.
Basophils and eosinophils increased
Cancer of the lymph tissue that happens when B or T cells divide faster than normal and live longer than normal
Two main types of lymphoma
Non-Hodgkin lymphoma (MC)
Risk factors for lymphoma
- exposure to virus (e.g. hepatitis B, C, Epstein-Barr)
- exposure to Helicobacter pylori
- exposure to chemical (e.g. herbicides) and radiation
- autoimmune diseases (e.g. Sjögren syndrome, SLE)
Clinical manifestation of lymphoma that is characterized by
-painless enlargement of nodes
-rubbery and swollen when examined
-nodes of the cervical, supraclavicular, chest, groin, and across whole body
DDx between lymphomas:
1. One type
2. Begins w/ B cells
3. Age 15-35 &over 50
4. MC in Females
5. Lymphadenopathy begins in upper body and spreads to lower
6. Lymphnodes painful after alcohol consumption
7. In biopsy of lymphnodes: Reed-Steinberg cells found
Reed Sternberg cells are described as ?
Found in hodgkins lymphoma
LArge often bi-uncleared cells with prominent nucleoli, originating from B-lymphocytes
Hereditary clotting factor disorders
Von willebrand disease
Acquired clotting factor disorders
Disseminated intravascular coagulation
Von Willebrand disease is described as?
The most common hereditary coagulation abnormality described in humans, developed through von willebrand factor deficiency
Von willebrand disease results in
Non formation of platelet plus and excessive bleeding
Von willebrand disease is a/w an autosomal dominant inheritance of which chromosome
12- always one parent with disease
Clinical manifestation of vWD
Recurring GI bleeding
Intensive menstruation hemorrhage (menorrhagia)
Prolonged bleeding and unaffected platelet count
Hemophilia is described as?
Rare genetic disorder that prevents the blood from clotting properly due to lack of clotting factors 8, 9, & 11
Types of hemophilia and their associated factor
Hemophilia A- VIII (80% of cases)
Hemophilia B/Christmas dz-lack of CF 9
Hemophilia C- deficiency of factor 11
T/F: Hemophila affects both genders equally
Hemophilia patients have parents that are affected/unaffected by bleeding disorder
Clinical manifestation of hemophilia
Usually discovered before 18 months old
Bleeding larger in weight bearing joints—>hemarthrosis
Bleeding tongue may close airway
Disseminated intravsacular coagulation is described as
Disorder in which CF become overreactive resulting is small clots in small vessels in the entire body
Explain whether or not DIC is a primary or secondary disorder.
DIC is a secondary disorder due to the fact it accompanies an underlying condition usually with critical illness
Clinical manifestation of DIC
Drop in BP
Dysfunction of all organs
Disorder where there is abnormally low platelets
Thrombocytopenia- regular platelet count should be between 150,000-450,000
Causes of thrombocytopenia
Increased platelet destruction
Various blood disorders
Clinical manifestations of thrombocytopenia
Rare disorder, causing extensive microscopic clots to form in the small blood vessels throughout the body
Thrombotic thrombocytopenic purpura
Five features of Thrombotic thrombocytopenic purpura
Microangiopathic hemolytic anemia
A disorder that usually occurs when an infection in the digestive system produces toxic substances that:
cause kidney injury
form blood clots in small blood vessels
Hemolytic-uremic syndrome (one of the TTP)
What is the common causative factor of hemolytic uremic syndrome
Post GI infection with E.coli