Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

OMS Board Review > 16. Hematologic Disease > Flashcards

16. Hematologic Disease Flashcards

1
Q

Define Anemia

What are normal values for hemoglobin and hematocrit for males and females?

A

Anemia is defined as a decrease in red blood cells (RBC) leading to less oxygen carrying capacity and delivery to end organs.

Normal Hemoglobin 14-18g/dL males, 12-16g/dL females

Normal Hct 40-52% males, 35-47% females

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Causes of anemia

A

Disorders of bone marrow production, red cell maturation, increased destruction, and iron deficiency.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

How is anemia subclassified?

A

Microcytic
Normocytic
Macrocytic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Microcytic Anemia

A

MCV < 80

Iron deficiency
Thalassemia
Sideroblastic anemia
Lead poisoning

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Normocytic Anemia

A

MCV 80-100

Acute blood loss
Hemolysis
Anemia of chronic disease
Anemia of renal failure
Myelodysplastic syndromes
Aplastic anemia
Hemolytic anemias

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Macrocytic Anemia

A

MCV >100

Folate deficiency
Vitamin B12 deficiency
Drug toxicity
Alcoholism/chronic liver disease

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What does a reticulocyte count tell you?

A

Reticulocyte = immature erythrocyte (new RBC that is 1-1.5 days old)

Reticulocyte count = fraction of RBCs consisting of reticulocytes that indirectly indicates the bone marrow activity of the erythrocyte line; expressed as a percentage (normal value 1%)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Iron Deficiency Anemia (definition, causes)

A

Iron deficiency anemia results from decreased synthesis of heme. Most common cause of anemia in US. Microcytic anemia.

Blood loss, increased utilization (pregnancy, infants)

Decreased intake (infants, elderly)

Decreased absorption (celiac sprue)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Treatment of iron deficiency anemia

A

Iron supplementation
Transfusion for severe cases
Treat underlying disease process

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Thalassemia Anemias (definition, causes)

A

Thalassemia Anemias are a group of autosomal recessive anemic disorders due to the abnormal production of globin chains. Microcytic anemia.
- Alpha thalassemia
- Beta thalassemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Alpha thalassemia

A

Decrease in synthesis of the alpha-globin chain of hemoglobin.
- Southeast Asia and Black Americans
- Four genes control production of alpha chain of Hgb; combinations of deletions of 1, 2, 3, or 4 cause different types and degrees of alpha-thalassemia
- One or two deletions = mild anemia, little symptoms, no treatment
- 3 gene deletions = HbH or four beta-chain disease due to macrophage destruction of RBCs due to excess beta chains
- 4 gene deletions = incompatible with life

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Beta thalassemia

A

Decrease in synthesis of beta-globin chain of Hgb
- Common in Black Americans, Greeks, Italians
- Beta thalassemia minor: mild microcytic anemia due to DNA splicing defect, decrease in HbA, increase in RBC count, HBA2 and ferritin production (no treatment)
- Beta thalassemia major: severe hemolytic anemia due to nonsense mutation with formation of stop codon; no production of HbA, increased production of HbA2 and HbF; lifelong danger of iron overload and lifelong need for transfusion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Most common cause of macrocytic anemia

A

Vitamin B12 or folate deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Most common cause of B12 deficiency

A

Pernicious anemia (decrease in intrinsic factor due to autoimmune destruction of gastric parietal cells).

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Most common cause of folate deficiency

A

Alcoholism

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Characteristic difference between Vit B12 and Folate deficiency

A

Neurologic disease is seen in B12 deficiency but not folate deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Most common anemia in hospitalized patients

A

Anemia of chronic disease (normocytic)

Chronic inflammation (rheumatoid arthritis, TB), alcoholism, malignancy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Most common cause of autoimmune hemolytic anemia

A

SLE (systemic lupus erythematosus)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Treatment of hereditary spherocytosis

A

splenectomy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What is sickle cell anemia?

A

Autosomal recessive disorder - intrinsic defect causing a hemoglobinopathy that causes extravascular hemolysis of sickled RBCs
- Most common hemoglobinopathy in patients of African descent
- Missense point mutation valine is substituted for glutamic acid at the sixth position of the beta-globin chain of Hb; causes sickling of RBCs.
- Key pathologic processes = severe hemolytic anemia, vaso-occlusive crises.
- Management with fluid hydration, pain management, oxygen therapy and steroids
- Treatment with hydroxyuria (increases HbF which prevents sickling and bone marrow transplant).

21
Q

What are some clinical findings and complications of sickle cell anemia?

A

Aplastic crisis (parvovirus B19 infection)
Dactylitis (swelling of hands and feet in infants)
Acute chest syndrome (vaso-occlusion of pulmonary capillaries; high morbidity)
Aseptic necrosis of femoral head
Autosplenectomy (accumulation of sickled cells)
Increased risk of infection from encapsulated organsims (Strep pneumonia, Neisseria meningitidis, Haemophilus influenzae type b)
Pain crises (vaso-occlusion)
Splenic sequestration crisis

22
Q

What is leukemia?

A

Leukemia is a disease of the mesencymal cells of the blood in which there is an abnormal proliferation or an increased lifespan of myeloid or lymphoid cells

23
Q

General characteristics of all leukemias

A
  • Increased number of circulating leukocytes in the blood.
  • Infiltration of bone marrow by leukemic cells (both mature and immature)
  • Most common sites of leukemic infiltration are spleen, liver, lungs, skin, lymph nodes
  • Bone marrow failure: anemia (decreased RBCs), granulocytopenia of mature WBCs (leading to infections), and thrombocytopenia (decreased platelets) leading to hemorrhage.
24
Q

Types of leukemias

A

Myeloid leukemias
- Acute myelogenous leukemia (AML)
- Chronic myelogenous leukemia (CML)

Lymphoid leukemias
- Acute lymphocytic leukemia (ALL)
- Chronic lymphocytic leukemia (CLL)

25
Q

Acute Myelogenous Leukemia (AML)

A

AML: incidence increases with age, most commonly 15-59. 80% of leukemia in adults. Men>women.

Hereditary, environmental, and drug related risk factors

Fatigue, gingivitis, bleeding and bruising, splenomegaly.

CBC shows anemia and thrombocytopenia.

Treatment = anthracycline drug (idarubicin or daunorubicin) and antimetabolite cytosine arabinoside (Ara-C).

26
Q

Risk factors for development of AML

A

Hereditary: Fanconi’s anemia, Down syndrome, Klinefelter’s syndrome, Patau’s syndrome, Neurofibromatosis.

Environmental: Benzene, Petroleum, Radiation

Drugs: Alkylating agents: busulfan, cisplatin, carboplatin, chlorambucil, cyclophosphamide.

27
Q

Chronic Myelogenous Leukemia

A

CML: malignant myeloproliferative disorder associated with Philadelphia chromosome. Rare in children. Older adults >60.

Risks: ionizing radiation and benzene.

Indolent course, incidentally discovered on blood analysis.

Signs of anemia, fatigue, petechiae, mucosal bleeding.

Treatment: tyrosine kinase inhibitors (imatinib, dasatinib, nilotinib, bosutinib. Interferon-alpha as second line. Chemotherapy with stem cell transplant.

28
Q

Acute Lymphocytic Leukemia

A

Malignancy characterized by proliferation of immature lymphoid cells in peripheral blood, bone marrow, and other organs.
- Can be lymphocytic B-cell, T-cell, or NK-cell neoplasm (85% B cell)
- Most common childhood leukemia
- Infection, fatigue, pallor, spontaneous bleeding (oral)
- Most responsive to therapy: tyrosine kinase inhibitors for Philadelphia chromosome-positive disease in addition to vincristine, daunorubicin, prednisone, and cyclophosphamide.

29
Q

Chronic Lymphocytic Leukemia

A

Lymphoproliferative disorder with proliferation and accumulation of mature-appearing neoplastic B-cells that cannot differentiate into plasma cells.

Most common overall leukemia (adults >60 typically)

Indistinct clinical presentation, up to 70% of patients asymptomatic.

“B symptoms” fever, night sweats, weight loss
Frequent infections, bleeding tendencies.

No standard curative treatment, goal is for prolongation of life and symptom relief.

30
Q

What is hemophilia?

A

X-linked recessive bleeding disorder (two types, A and B)

Hemophilia A: congenital deficiency in clotting factor VIII. Results from factor 8 gene mutations on the X chromosome. X-linked recessive inheritance pattern. Males > Females.

Hemophilia B: congenital deficiency in clotting factor IX. Results from factor 9 gene mutations on the X-chromosome. X-linked recessive inheritance pattern. Males > Females.

31
Q

Diagnosis of hemophilia

A

Genetic testing if known family history in utero.
Mild disease may not be diagnosed until major trauma or surgery as a young adult.

Partial thromboplastin time is prolonged
Prothrombin time is normal
Platelet count is normal

Hemophilia A: factor VIII activity level below 40% of normal
Hemophilia B: factor IX activity level below 40% of normal.

32
Q

Clinical features of hemophilia

A

Clinical features of A and B are very similar. Classification is based on severity of disease.

Severe disease: factor activity levels below 1% of normal. Spontaneous bleeding multiple times per year

Moderate disease: factor activity levels between 1-5% of normal. Infrequent spontaneous bleeding but prolonged bleeding as a result of trauma or surgical procedures occurs.

Mild disease: factor levels greater than 5%, but below normal. Prolonged bleeding with trauma or surgical procedures.

Hemarthrosis can result in joint damage
Intracranial hemorrhage in newborns
Muscle bleeding, GI bleeding, hematuria, easy bruising
Compartment syndrome.

33
Q

Treatment of hemophilia

A

Patient education, avoid contact sports
Avoid NSAIDs and ASA
Regular orthopedic eval with joint involvement

Recombinant factor concentrates of VIII or IX. Goal to raise levels 80-100% prior to surgery and maintain 50% 1-2 weeks postoperatively.
Factors administered 20 mins postoperatively due to short half-lives.

Hemostatic adjuncts to factor concentrate admin.
Antifibrinolytic agents such as tranexamic acid
Desmopressin (DDAVP): IV, subq, or intranasal. Can be used for mild to moderate hemophilia A as it increases FActor VIII:C and vWF levels. Avoid excess water intake to prevent hyponatremia.

34
Q

What is Von Willebrand Disease?

A

Congenital platelet bleeding disorder due to deficiency or dysfunction of von Willebrand Factor (vWF).

vWF is responsible for mediating platelet adhesion, adhesion to endothelium, and prevention of degradation of Factor VIII.

Most commonly inherited bleeding disorder (not sex-linked). Autosomal dominant inheritance pattern is most common.

Can also be acquired by hematoproliferative disorders or in autoimmune disorders such as SLE or hypothyroidism

35
Q

vWD is divided into three types

A

Type 1: partial quantitative deficiency (most common type)
Type 2: qualitative deficiency (further divided into different subtypes 2A, 2B, 2N, 2M)
Type 3: total or near total quantitative deficiency (autosomal recessive).

36
Q

How is vWD diagnosed?

A

Note that no single laboratory test is available, recommendation for three assays for initial screening
- Ristocetin cofactor assay (ability of patient plasma to agglutinate platelets by addition of antibiotic Ristocetin (off market due to ability to cause thrombocytopenia and platelet agglutination).
- vWF antigen (see decrease)
- Factor VIII coagulant activity (see decrease)

CBC, PT normal
PTT normal unless factor 8 is affected.

37
Q

Treatment of vWD

A

Type 1: DDAVP (IV or intranasal)
Type 2 and 3: DDAVP in some type 2 variants can cause an increase in abnormal vWF and is often avoided. Cryoprecipitate can be used to treat all types of vWD. Contains factor 8, 9, vWF, fibrinogen, fibronectin. Usually reserved to treat qualitative forms.
- Factor VIII concentrate is rich in vWF
- Humate-P (vWF/Factor VIII) concentrate
- Amicar: antifibrinolytic agent given orally or IV

38
Q

Antithrombin III deficiency

A

Inherited deficiency of antithrombin III leading to increased risk for recurrent thrombosis (DVT and PE).
- Concern for heparin resistance
- Can be quantitative or qualitative
- Treated with heparin and bridged to warfarin if h/o thrombosis
- May require FFP, cryoprecipitate, or AT III concentrate perioperatively

39
Q

Protein C Deficiency

A

Qualitative or quantitative deficiency in protein C, which inhibits blood coagulation by inactivation of factors 5 and 13 causing a prothrombotic state.
- Protein C is a Vit K dependent coagulation factor synthesized in the liver.
- Can be inherited or acquired (liver damage, DIC, vit K deficiency).
- Treatment with warfarin if history of thrombosis
- Acute thrombosis may require heparin and/or transfusion of FFP to increase protein C levels.

40
Q

Factor V Leiden

A

Hereditary gene mutation resulting in resistance to activated protein C.
- Resistance to inactivation of factor V by activated protein C secondary to a F5 gene mutation.
- Heterozygous: 5-10 fold increased risk for thrombosis.
- Homozygous: 50-100 fold increased risk for thrombosis.

DVTs, pulmonary embolism, superficial thrombophlebitis.

Infants may present with thrombotic purpura or purpura fulminans and disseminated intravascular coagulation.

  • Treat with early ambulation after surgery, mechanical DVT prophylaxis. Subq heparin may be warranted.
  • Long-term anticoagulation with warfarin is sometimes indicated.
41
Q

Lymphoma

A

Malignancy of lymphocytes that usually arises in lymph nodes.
- Hodgkin
- non-Hodgkin

42
Q

Hodgkin lymphoma

A

Malignancy of lymphocytes that usually arises in lymph nodes.
- Male predilection for HL
- Painless lymphadenopathy (supraclavicular, 75% in neck)
- B symptoms- night sweats, fevers, weight loss
- Spleen involved 20% of the time
- Extranodal involvement in bone marrow, lungs, liver, and bone
- Presence of Reed Sternberg malignant cells (binucleated cells with owl eye nuclei) on histopath.
- Work-up = biopsy, imaging (CT/PET), CBC, CMP (anemia, leukocytosis, thrombocytosis, hypoalbuminemia).

Treatment: external beam radiation 20Gy. Chemotherapy (adriamycin, bleomycin, vinblastine, dacarbazine)

43
Q

Non-hodgkin Lymphoma

A
  • Malignant lymphoproliferative lesions that are not Hodgkin lymphoma
  • 85% are of B-cell origin
  • B symptoms and painless lymphadenopathy
  • Extranodal involvement in bone marrow, lungs, liver, bone.
  • Low, intermediate, or high grade
  • Cells express CD20 marker
  • Viruses may play a role (EBV - Burkitt’s lymphoma, HHV, HTLV, HIV, Hep B)
  • Workup with histopath, imaging, CBC/CMP (anemia, leukocytosis, thrombocytosis, hypoalbuminemia.
  • HIV/Heb B serological assays

Treatment: external beam radiation, Rituximab (anti CD20 monoclonal antibody), CHOP chemotherapy (cyclophosphomide, doxorubicin, vincristine, prednisone), stem cell transplant

44
Q

Multiple Myeloma

A

Malignancy characterized by the neoplastic transformation of plasma cells producing a monoclonal immunoglobulin.
- Proliferation in bone marrow resulting in osteolytic lesions, hypercalcemia, osteopenia, and pathologic fractures
- Older adults (66yrs)
- African descent 2-3x whites
- Bone pain, anemia, increase urine monoclonal protein, increase total serum protein, renal failure, hypercalcemia due to osteolysis, susceptibility to infection.

Workup with protein electrophoresis to detect monoclonal protein, serum and urine M protein. Peripheral smear shows Rouleaux formation (stacked RBC resembling coins), leukopenia, thrombocytopenia. Bone marrow biopsy used to assess for myeloma cells. CBC, CT, PET.

Treatment with external beam radiation, VRD chemotherapy (velcade, revlimid, decadron). Stem cell transplant. Antiresorptive agents for lytic lesions (bisphosphonates and denosumab).

45
Q

Lab tests for vWD

A
  • Plasma vWF levels (ristocetin cofactor activity assay used to measure platelet aggregation): may vary with stress, hormone replacement, pregnancy
  • Plasma vWF antigen can be normal
  • Factor VIII activity
46
Q

5 treatment modalities for treatment of vWD

A
  1. Desmopressin (DDAVP)
  2. vWF replacement therapy (using cryoprecipitate)
  3. Antifibrinolytic agents
  4. Topical agents (thrombin or fibrin sealants)
  5. Estrogen therapy (in women with no contraindications)
47
Q

What is DDAVP?

How does it work? How is it administered?

A

A synthetic analog of antidiuretic hormone without vasopressor activity.

Increases vWF and factor VIII levels by indirectly stimulating release of vWF from endothelial cells.

Can be given IV, IM, or IN

Increase in vWF and factor VIII seen within 30-60 minutes with duration of 6-12 hours.

Should NOT be given to patients with type 2B vWD because it may worsen the disease.

48
Q

vWF replacement therapy

A

For cryoprecipitate (which contains factor VIII) to contain viable vWF, it cannot be pasteurized, only screened (increased risk of viral transmission).

Most factor VIII concentrates do not contain sufficient high-molecular-weight vWF

Drugs Humate-P and Alphanate do contain sufficient amounts.

49
Q

Types of vWD

A

Type 1: AD, most common (70%), quantitative deficiency in vWF itself. Mild to moderately severe. Deficiency may be from abnormally fast clearance of the protein or inadequate production.

Type 2: AD, qualitative abnormality. 2A, 2B, 2M, 2N. (2B - DDAVP may actually exacerbate the condition removing more platelets from circulation)

Type 3: rare. Complete absence or very low levels of vWF. Severe bleeding.

OMS Board Review (29 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions