Chapter 3 Hematologic MDT Flashcards
Hematocrit in adult males less than 41% (hmg < 13.5g/dL)
Hematocrit in adult females less than 37% (hmg <12 g/dL)
Anema
Commonly the cause of iron deficiency in adults
Bleeding
Two anemia classifications
Pathophysiologic basis (increase or decrease production of RBCs)
Cell size
Signs and symptoms:
Lymphadenopathy, hepatosplenomegaly, or bone tenderness
Mucosal changes such as a smooth tongue
Anemia (megaloblastic)
Anemia labs:
Iron with total iron binding capacity (TIBC)
-Less than what ferritin value indicates Fe deficiency anemia?
12 mcg/L
Anemia labs:
Why would you get hemoglobin electrophoresis?
To evaluate for alpha or beta thalassemia
Treatment for:
Anemia
Identification of blood loss
Treatment specific to the specific cause of anemia
Most common cause of anemia
Iron deficiency
Increases the chances of iron deficiency anemia
Menstruation
Pregnancy
Frequent blood donors
Most important cause of iron deficiency anemia
Blood loss, especially GI blood loss
Physical findings:
Fatigability, tachycardia, palpitations, and tachypnea on exertion
Severe: Skin/mucosa changes. Smooth tongue, brittle nails, cheilosis
Iron deficient anemia
Cause of dysphagic in iron deficient anemia patients
Formation of esophageal webs (Plummer-Vinson syndrome)
Many iron-deficient patients develop this, crave specific foods often not rich in iron
Pica
Labs for iron deficient anemia
CBC - decreased mean corpuscular volume (MCV)
Iron - Ferritin value <12 mcg/L
Treatment for iron deficient anemic patients
Ferrous sulfate 325mg TID for 3-6 months
Vitamin B12 deficiency can cause
Macrocytic anemia
-B12 level <100 pg/ml
All vitamin B12 comes from:
Diet, all foods of animal origin
The daily absorption of vitamin B12
5 mcg
Liver contains ____-____ mcg of stored vitamin B12.
Daily losses are ___ mcg/dL.
The body has enough B12 stores for __ years.
2000-5000mcg
3-5 mcg/dL
3 years
Surgical resection of the ileum will eliminate the site of:
B12 absorption
Rare causes of B12 deficiency
Fish tapeworm infection
Pancreatic insufficiency
Severe Crohn’s disease (destroys the ileum)
Physical findings:
- Glossitis
- Anorexia
- Diarrhea
- Late stages: Pale skin, paresthesia and difficulty with balance
B12 deficiency
Hallmark lab findings in B12 deficiency
CBC w/ Diff: Megaloblastic Anemia (Large RBCs)
-Macro-ovalocyte with hyper-segmented neutrophils
B12 deficiency:
Mean corpuscular volume (MCV) will be:
Strikingly elevated (MCV = RBC volume from CBC)
Treatment for B12 deficiency
IM Injection of B12
- Daily first week
- Weekly first month
- Monthly for life
Three mechanisms that reduce loss of blood from blood vessels
Vascular spasm
Platelet plug formation
Blood clotting
Vascular spasm is caused by damage to smooth muscle and reflexes initiated by _____ receptors
Pain
Three stages of blood clotting
Formation of prothrombinase
Conversion of prothrombin into thrombin
Thrombin converts soluble fibrinogen into insoluble fibrin threads
Factors that are vitamin K dependent
II
VII
IX
X
Laboratory measurement of the function of intrinsic and extrinsic coagulation pathways
PTT (intrinsic)
PT (extrinsic)
Congenital deficiency of coagulation factor VIII
Hemophilia A
Congenital deficiency of coagulation factor IX
Hemophilia B
In many older patients, what viruses from receipt of contaminated blood products can cause coagulopathy?
HIV
Hep C
Life threatening emergency.
Causes thrombosis and hemorrhage.
Coagulation and fibrinolysis become abnormally activated, leading to ongoing coagulation and fibrinolysis.
Disseminated intravascular coagulation (DIC)
Common bleeding manifestations of DIC
Petechiae
Ecchymoses
Blood oozing from wound sites, IV lines, catheters, mucosal surfaces
Common thromboembolic manfestations of DIC
Venous thromboembolism (VTE) and arterial thrombosis with tissue or organ ischemia
Common causes of DIC
Sepsis
Malignancy
Trauma
OB complications
Hemolysis (malaria or ABO incompatible transfusion)
Less common causes of DIC
Heat stroke
Crush injuries
Rattlesnake/Viper Bite
Treatment for DIC
Treat the underlying cause
Symptoms/physical findings:
- Bruising without trauma
- Bleeding into joint spaces
- Epistaxis
- Bleeding from eyes
- Very heavy vaginal bleeding for prolong times in females
DIC
Labs:
DIC
PT, PTT
X-linked recessive disorder commonly seen in American black men, affecting 10-15%
Episodic hemolysis in response to oxidant drugs or infection
G6PD
G6PD
Glucose-6-phosphate dehydrogenase
G6PD:
Oxidized hemoglobin denatures and forms precipitants called:
Heinz bodies
G6PD patients to avoid oxidant drugs like:
Dapsone
Primaquine
Quinidine
Quinine
Sulfonamides
Nitrofurantoin
Aspirin
Ciprofloxacin
Labs for G6PD:
Red blood cell smear is not diagnostic but may reveal a small number of “____” cells
“bite”
G6PD:
Support by monitoring with what lab test?
CBC
Autosomal recessive disorder in which an abnormal hemoglobin leads to chronic hemolytic anemia with numerous clinical consequences
Sickle Cell
The rate of sickling is influenced by the concentration of _______ and by the presence of other hemoglobin within the cell
Hemoglobin S
Hemoglobin S gene is carried in __% of American black people
8%
_ of 400 American black children will be born with sickle cell anemia
1 out of 400
Symptoms/Physical findings:
Chronically produces jaundice, pigment gallstones, hepatosplenomegaly, poorly healing ulcers over the lower tibia.
Life-threatening anemia can occur during hemolytic/aplastic crises, usually associated with viral or other infection or by folate deficiency
Acute painful episodes include the bones and chest, last hours to days and may produce a fever
Cardiomegaly
Chronic pain
Sickle Cell Anemia
Lab studies:
Sickle Cell
CBC w/ Diff
-Hematocrit is usually 20-30%
Sickel Cell:
Blood smear will show what percentage of irreversibly sickled cells
5-50%
SCA:
Hallmarks of hypersplenism
Howell-Jolly bodies and target cells
Sickle cell hemoglobin is confirmed by hemoglobin electrophoresis with hemoglobin S accounting for __% of hemoglobin.
86-98%
Disease where no hemoglobin A is present
Homozygous S disease
Allogeneic hematopoietic stem cell transplantation before end organ damage, it can cure __% of children with SCA.
80%
Treatment for SCA:
Folic acid supplements (1mg PO daily)
Transfusions for aplastic/hemolytic crises
Keep hydrated
Search for underlying infection
Average life expectancy for SCA is:
40-50 years old
Sickle cell trait
Heterozygous genotype (AS)
Sickle cell trait:
Screening for sickle hemoglobin will be positive.
What percentage will be hemoglobin S?
40%
Malignancy of the hematopoietic progenitor cell. These cells proliferate in an uncontrolled fashion and replace normal bone marrow.
Leukemia
Leukemia is linked to:
Radiation and some toxins (benzene)
Compromised 80% if the acute leukemias of childhood. It is seen in adults, causing approximately 20% of adult acute leukemias.
Acute lymphoblastic leukemia (ALL)
Primarily an adult disease with a median age at 60 years and an increasing incidence with advanced age.
Acute myeloid leukemia (AML)
Leukemia:
Blasts in peripheral blood in __% of patients
90%
Symptoms/Physical findings:
- Fatigue
- Bleeding (gums, mucosa, skin)
- Infection
- Gum hypertrophy, bone and joint pain
- Pale skin and have purpura and petechiae
- Enlargement of liver, spleen, and lymph nodes
- Bone tenderness, particularly in the sternum, tibia, and femur
Leukemia
Hallmark of acute leukemia is:
Combination of pancytopenia with circulating blasts
Aleukemic leukemia
No presence of blasts (10% of cases)
What percentage of blasts require to make a diagnosis of acute leukemia??
20%
Patients with Acute Lymphoblastic Leukemia may show what on chest radiograph?
Mediastinal mass
Treatment for leukemia
Referral to hematologist
MEDEVAC
Chemo and radiation therapy is the mainstay
Approximately ___% of adults with AML under age 60 y/o achieve complete remission
70-80%
Chemotherapy leads to the cure in __% of patients
35-40%
Bone marrow transplants is curative in ___% of cases
50-60%
High WBC count
Leukocytosis
Low WBCs (<4400 cells/mL)
Leukopenia
WBC:
- Bacterial infection
- Inflammation
- Metabolic disease
- Stress
Neutrophils
WBC:
- Viral infection
- Immune disease
- Stress
- Leukemia
Lymphocytes
WBC:
- Skin diseases
- Drug reaction
- Parasite infection
- Asthma
Eosinophils
WBC:
-Chronic myeloid leukemia
Basophils
WBC:
- Infection
- Autoimmune disease
Monocytes
Benign ethnic neutropenia
Infections (Hepatitis, HIV, Epstein-Barr, Parasitic, rickettsia infections)
Medications
Nutrition: vitamin deficiencies (B12, folate, copper)
Hematologic malignancies (leukemia, lymphoproliferative disorders, myelodysplastic syndromes)
Rheumatic disorders (RA, SLE)
Etiologies of leukopenia
Signs and symptoms of leukopenia or leukocytosis
Fever
Skin erythema, ulcerations, fissures, or tenderness
Gingivitis, swelling, oral ulceration, dental pain
Abnormal respiratory exam
Neuro or psychiatric abnormalities may suggest what?
Nutritional deficiency (B12, copper)
Abnormally low amount of circulating platelets
Thrombocytopenia
The risk of spontaneous bleeding does not typically increase until the platelet count falls below ____/mcL
10,000-20,000
Potential causes of thrombocytopenia:
Bone marrow failure or malignancy
Disseminated intravascular coagulation (DIC)
Chemo or radiation therapy
Nutritional deficiencies (folate or iron)
Medications
Potential causes of thrombocytopenia:
Bone marrow failure or malignancy
Disseminated intravascular coagulation (DIC)
Chemo or radiation therapy
Nutritional deficiencies (folate or iron)
Medications
Signs and symptoms of thrombocytopenia
PETECHIA (coagulopathy would cause bruising)
Mucocutaneous bleeding (gingival, nosebleeds)
Abnormally high amount of circulating thrombocytes (>450,000)
Thrombocytosis
Two different categories of thrombocytosis
Reactive
- Anemia/blood loss
- Infection
- Non-infectious inflammation
- Post splenectomy
Autonomous
-Primarily cancer of bone marrow
Signs/Physical findings:
- First sign is elevated platelets
- 50-60 y/o
- Erythromelalgia - painful burning of the hands w/ erythema
- Splenomegaly in 25%
Thrombocytosis
Labs/Studies:
Thrombocytosis
CBC w/ diff
Blood smear
Iron studies
Inflammatory markers (based on history)
Treatment:
Thrombocytosis
Refer to hematology if there is no infectious cause or it does not resolve on its own in 2-4 weeks