CLINICAL CARE OF THE HEMATOLOGIC SYSTEM Flashcards
True/false
Congenital anemia is suggested by the patient’s personal and family history.
True
Poor _____ may result in folic acid deficiency and contribute to iron deficiency, but bleeding is much more commonly the cause of iron deficiency in adults.
diet
Anemia is defined by a hematocrit in adults less than ______ in males or less than _____ in females.
41% (hemoglobin 13.5 g/dL) in males
37% (hemoglobin less than 12 g/dL) in females.
Anemias are classified according to either:
- Pathophysiologic basis, i.e., whether related to increased or decreased production of red blood cells
- Cell Size
Mucosal changes such as a smooth tongue suggest ________
Megaloblastic anemia
S/s for Anemia
Attention to signs of primary hematologic diseases such as
lymphadenopathy,
hepatosplenomegaly, or bone tenderness
Labs to run for suspected anemia
-
(1) CBC with differential*
(2) Iron with total iron binding capacity (TIBC)
a) Ferritin value <12 mcg/L indicates Fe deficiency anemia
(3) Microscopic analysis
(4) Hemoglobin electrophoresis (to evaluate for alpha or beta thalassemia)
TX for anemia
(1) Most important treatment is identification of the cause of blood loss, especially a
source of occult blood loss.
(2) Other treatment specific once cause of anemia is identified
_____ is the most common cause of anemia worldwide.
Iron deficiency
True/False
Menstruation, pregnancy, and frequent blood donors increase chances for iron deficiency anemia.
True
Prolonged aspirin use, or the use of other anti-inflammatory drugs, may cause _____ even without a documented structural lesion.
GI bleeding
All vitamin B12 comes from the diet and is present in all foods of animal origin. The daily absorption of vitamin B12 is ___
5 mcg
vitamin B12 deficiency develops more than ____ after vitamin B12 absorption ceases
3 years
- What Dx/ TX
1) Glossitis
2) Anorexia
3) Diarrhea
4) Late stages patient will appear pale, with paresthesia and difficulty with balance. - Lab/Image Findings
1) CBC with diff: Hallmark is megaloblastic anemia (large RBC’s), characteristic find is macro-ovalocyte with hypersegmented neutrophils.
2) Mean corpuscular volume (MCV) strikingly elevated. MCV= RBC volume as part of CBC w/diff
3) Abnormally low B12 serum level. Overt deficiency <170 pg/mL, Symptomatic <100 pg/mL
4) Elevated LDH and bilirubin
Vitamin B12 Deficiency Anemia
-Tx
IM injection of vitamin B12. Daily for first week, weekly for first month,
then monthly for life.
True/False
B-12 deficiency is a Lifelong disease, deficiency will recur if injections discontinued
True
What Dx/Tx
1) Fatigability, tachycardia, palpitations and tachypnea on exertion.
2) Severe deficiency causes skin and mucosal changes, including a smooth tongue, brittle nails, and cheilosis.
3) Dysphagia because of the formation of esophageal webs (Plummer- Vinson syndrome) also occurs.
4) Many iron-deficient patients develop pica, craving for specific foods (ice chips, etc) often not rich in iron.
- Lab/Image Findings
1) CBC with differential
a) Decreased mean corpuscular volume (MCV).
2) Iron with total iron binding capacity (TIBC)
3) Ferritin value <12 mcg/L
Iron Deficiency Anemia
1) Most important treatment is identification of the cause of blood loss, especially a source of occult blood loss.
2) Oral Iron
Coag Intrinsic pathway, with key factors
XII, XI, VIII, IX.
Coag Extrinsic pathway with the key factor
VII
_________ is a systemic process with the potential
for causing thrombosis and hemorrhage. It can present as an acute, life-threatening
emergency.
Disseminated intravascular coagulation (DIC)
Laboratory measurement of the function of:
1) Intrinsic pathway is by measuring _____,
2) extrinsic is measured by ____.
1) PTT
2) PT
What issue
Common bleeding manifestations include petechiae; ecchymoses; and blood oozing from wound sites, intravenous lines, catheters, mucosal surfaces while Common thromboembolic manifestations of
DIC include venous thromboembolism (VTE) and arterial thrombosis with tissue or organ ischemia.
DIC
What dx/ TX
(1) Bruising (ecchymosis) without any known incident/trauma
(2) Bleeding into joint spaces.
(3) Epistaxis
(4) Bleeding from eyes
(5) Very heavy vaginal bleeding for prolonged periods of times in females.
DIC
- The major principle in the management of DIC is treatment of the underlying cause in order to eliminate the stimulus for ongoing coagulation and thrombosis.
- This is true medical emergency and requires immediate MEDEVAC
Meds to consider for DIC
(a) Heparin
(b) Coumadin
(c) Rivaroxaban
(d) Apixaban
(e) Pradaxa
Labs to run for DIC
(1) PT, PTT, INR
(2) CBC
______ is a hereditary enzyme defect that causes episodic hemolytic anemia because of the decreased ability of red blood cells to deal with oxidative stresses.
Glucose-6-phosphate dehydrogenase (G6PD) deficiency
_____ is the process of RBCs rupturing
Hemolysis
Pertinent physiology for what issue
(1) X-linked recessive disorder seen commonly in American black men, affecting 10-
15%
(2) Episodic hemolysis in response to oxidant drugs or infection
(3) Minimally abnormal peripheral blood smear
(4) Reduced levels of glucose-6-phosphate dehydrogenase between hemolytic episodes.
G6PD deficiency
Symptoms/Physical Exam Findings of what issue
(1) Patients are usually healthy, without chronic hemolytic anemia or splenomegaly.
(2) Hemolysis occurs as a result of oxidative stress on the red blood cells, generated either by:
(a) Infection
(b) Exposure to certain drugs including:
1) Dapsone
2) Primaquine
3) Quinidine
4) Quinine
5) Sulfonamides
6) Nitrofurantoin
7) Aspirin
8) Ciprofloxacin
G6PD
True/false
In g6pdBetween hemolytic episodes, the blood is anemic.
False
Normal
Treatment considerations for G6PD
(1) No treatment is necessary except to avoid oxidants.
(2) Patient Education to avoid known oxidant drugs.
(3) Supportive by monitoring CBC
(4) Potential to require blood transfusion in severe cases.
(5) For military members ensure possession of red dog tags as well as warning on front
of health record.
(6) May need blood transfusion in severe cases
Sickle cell anemia
Abnormal RBC take the shape of a _____rather than a biconcave disc
sickle….
___________ is an autosomal recessive disorder in which an abnormal hemoglobin (Hemoglobin S) leads to chronic hemolytic anemia with numerous clinical consequences.
Sickle Cell Anemia
The hemoglobin S gene is carried in ____ of American blacks, and 1 of ____ American black children will be born with sickle cell anemia. Prenatal diagnosis is available for couples at risk for producing a child with sickle cell anemia.
8%
400
What issue/labs/Tx
(1) Chronic hemolytic anemia produces jaundice, pigment (calcium bilirubinate) gallstones, hepatosplenomegaly, and poorly healing ulcers over the lower tibia.
(2) Life-threatening severe anemia can occur during hemolytic or aplastic crises, generally associated with viral or other infection or by folate deficiency.
(3) Acute painful episodes include the bones (especially the back and long bones) and the chest. These episodes last hours to days and may produce low-grade fever.
(4) Cardiomegaly
(5) Non-healing ulcers of the lower leg and retinopathy may be present.
(6) Chronic pain
Sickle Cell Trait/Disease
-Labs
CBC/dif- Hematorcrit 20-30%
Blood smear: characteristically abnormal, with irreversibly sickled cells comprising 5-50% of red cells
-Tx
(1) When allogeneic hematopoietic stem cell transplantation is performed before onset of significant end organ damage, it can cure more than 80% of children with sickle cell anemia who have a suitable HLA matched donor. Transplantation in adults remains investigational.
(2) Supportive care is the mainstay of treatment.
(a) Patients are maintained on folic acid supplements (1mg orally daily)
(b) given transfusions for aplastic or hemolytic crises.
(3) When acute painful episodes occur, precipitating factors should be identified.
(a) Keep hydrated and prevent hypoxemia both of which are triggers
(b) Search for underlying infection (also another trigger for acute painful
episodes)
Sickle cell hemoglobin is confirmed by _______ with Hemoglobin S accounting for___% of hemoglobin. In homozygous S disease no hemoglobin A will be present
hemoglobin electrophoresis
86-98%
Sickle Cell
These persons are usually clinically normal but can have acute painful episodes only under extreme conditions such as __________
vigorous exertion at high altitudes (or in unpressurized aircraft).
_______ is a malignancy of the hematopoietic progenitor cell. These cells proliferate in an uncontrolled fashion and replace normal bone marrow elements.
Leukemia
Acute Leukemia
More than __% blasts in the bone marrow
20%
Acute lymphoblastic leukemia (ALL) comprises ___% of the acute leukemias of childhood. It is also seen in adults, causing approximately ___% of adult acute
leukemias.
80% Children
20% Adults
______________ is primarily an adult disease with a median age at presentation of 60 years and an increasing incidence with advanced age.
Acute myeloid leukemia (AML)
Leukemia
Blasts in peripheral blood in ___% of patients.
90
Vast majority of AML patients diagnosed in their __’s.
20’s
S/physical findings of what issue
(1) Most patients usually only complain about fatigue.
(2) Bleeding (usually due to thrombocytopenia) occurs in the skin and mucosal surfaces, with gingival bleeding, epistaxis, or menorrhagia.
(3) Infection due to neutropenia within days is the rule. Common presentations include cellulitis, pneumonia, and perirectal infections; death within a few hours may occur if treatment with appropriate antibiotics is delayed.
(4) Patients may also seek medical attention because of gum hypertrophy and bone and joint pain.
(5) On examination, patients appear pale and have purpura and petechiae; signs of infection may not be present.
(6) There is variable enlargement of the liver, spleen, and lymph nodes.
(7) Bone tenderness may be present, particularly in the sternum, tibia, and femur.
Leukemia
Differential for leukemia
- *Viral infection such as Mononucleosis**
- Pertussis
- Other 1 Myeloproliferative diseases:
(a) Hairy cell leukemia
(b) Lymphomas
(c) Chronic Lymphocytic Leukemia (CLL) - Anemia
- Hypothyroid
- SLE
The hallmark of acute leukemia is
the combination of pancytopenia with circulating
blasts.
More than ___% blasts are required to make a diagnosis of acute leukemia
20%
Patients with ____ may have a mediastinal mass visible on chest radiograph.
ALL
Bone marrow transplantation (for younger adults with HLA-matched siblings) is curative in _____% of cases.
50-60%
___________ is a high white cell count relative to normal physiologic numbers.
Leukocytosis
_______ is a low total white cell count (<4400 cells/microL).
Leukopenia
True/False
The different etiologies of leukocytosis can be determined based on the cell line that is elevated.
True
What leukocytosis?
(a) Bacterial infection
(b) Inflammation
(c) Metabolic disease
(d) Stress
Neutrophils (called Neutrophilia):
What leukocytosis?
(a) Viral infection (ex/ Bordetella pertussis, infectious mononucleosis, hepatitis).
(b) Immune disease
(c) Stress
(d) Leukemia
Lymphocytes (called Lymphocytosis)
What leukocytosis?
(a) Skin diseases
(b) Drug reaction
(c) Parasite infection
(d) Asthma
Eosinophils (called Eosinophilia):
Signs and Symptoms suggestive of what?
(1) Evidence of infection:
(a) Fever
(b) Skin erythema, ulcerations, fissures, or tenderness
(c) Gingivitis, swelling, oral ulceration, or dental pain
(d) Abnormal respiratory exam
(2) Other findings
(a) Jaundice
(b) Joint swelling or bone pain to suggest rheumatologic disorder,
infection, or malignancy.
(c) Rash may suggest a rheumatologic disorder or drug allergy.
(d) Lymphadenopathy or splenomegaly
(e) Neuro or psychiatric abnormalities may suggest a nutritional deficiency (B12, copper).
Leukopenia
True/False
Fore Leukocytosis and Leukopenia. CBC with differential will not determine the cell line that is being affected.
False
Will determine
________ is an abnormally low amount of circulating platelets.
Thrombocytopenia
The risk of clinically relevant spontaneous bleeding does not typically increase until the platelet count falls below ___________/mcL unless the patient has dysfunctional platelets.
10,000 - 20,000 /mcL
Symptoms/Physical Exam Findings for Thrombocytopenia
- PETECHIA: this helps you determine that the deficiency is platelets rather then a coagulopathy
- Mucocutaneous bleeding (i.e. gingival, nosebleeds).
Treatment of a patient with Thrombocytopenia (both pharmacologically and nonpharmacologically)
(1) Stop potentially offending medications.
(2) Evaluate for underlying cause
(3) Referral to Internal Medicine or Hematologist.
(4) MEDEVAC if actively bleeding.
______ is an abnormally high amount of circulating thrombocytes (>450,000).
Thrombocytosis
Causes of thrombocytosis are broken into 2 different categories
Reactive Thrombocytosis
Autonomous thrombocytosis
Reactive or Autonomous thrombocytosis? 1) Anemia or blood loss 2) Infection 3) Non-infectious inflammation (malignancy, rheumatologic conditions, trauma, reactions to medications). 4) Post splenectomy
Reactive Thrombocytosis
Reactive or Autonomous thrombocytosis?
primarily cancer of bone marrow etiologies.
Autonomous thrombocytosis
Symptoms/Physical Exam Findings for what issue
(1) Median age of presentation is 50-60 years of age.
(2) First sign is elevated platelets.
(3) Increased risk for thrombosis.
(4) Erythromelagia - painful burning of the hands accompanied by erythema.
(5) Splenomegaly in 25% of these patients.
Thrombocytosis
Treatment for Thrombocytosis
Refer to Hematology if there is no infectious cause or it does not resolve on its own in 2-4 weeks.
(1) Good history and physical examination.
(2) CBC and blood smear.
(3) If you cannot determine etiology of thrombocytosis then refer to Hematologist.
