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Pathophysiology > Hematology Disorders > Flashcards

Hematology Disorders Flashcards

1
Q

Leukopenia

A

Decrease in total number of leukocytes in the blood, most often affecting neutrophils

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2
Q

Agranulocytosis

A

absence of neutrophils

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3
Q

Aplastic anemia

A

All myeloid stem cells are affected, resulting in thrombocytopenia, agranulocytosis, and anemia.

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4
Q

Lab value for neutropenia

A
  • commonly defined as a circulating neutrophil count of less than 1500/μL
  • mild (1000 to 1500/μL)
  • moderate (500 to 1000/μL)
  • severe (<500/μL)
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5
Q

Cyclic neutropenia

A

autosomal dominant neutropenia begining in infancy, which occurs every ~21 days and lasts for 2-3 days

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6
Q

Kostmann Syndrome

A

Severe congenital neutropenia

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7
Q

What can cause acquired neutropenia?

A
  • Cancer
  • autoimmune disorders
  • Infection related
  • Felty Syndrome
  • Radiation to bones
  • Drug related (depresses bone marrow function)
  • Splenomegaly (neutrophils get trapped in the spleen)
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8
Q

What is Felty Syndrome?

A

A type of rhematoid arthritis in which neutrophils gets destroyed in the spleen

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9
Q

Common symptoms of neutropenia

A
  • Skin infections and ulcerative necrotizing lesions of the mouth
  • Respiratory tract infections
  • Pus is absent from infections because pus consists of tissue, WBCs, and pathogens that are liquified by neutrophil enzymes
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10
Q

Infectious mononucleosis

A

self-limiting lymphoproliferative disorder caused by the Epstein-Barr virus (EBV), a member of the herpesvirus family

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11
Q

Pathogenesis of mononucleosis

A
  • Spreads to oropharyngeal lymphoid tissue and to B lymphocytes, which have receptors for EBV
  • Infection may kill infected B cell, or virus may incorporate itself into the cell’s genome. The B cells with the EBV genome proliferate and produce heterophil antibodies (diagnostic test).
  • normal immune response is important in controlling proliferation of EBV-infected B cells with cytotoxic T cells and NK.
  • virus remains in a few transformed B cells in the oropharyn- geal region and is shed in the saliva. Once infected with the virus, asymptomatically infected for life
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12
Q

Incubation period for mononucleosis

A

4-8 weeks

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13
Q

Most common leukemia among children

A

Acute Lymphocytic Leukemia

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14
Q

Most common leukemias among adults

A

Chronic Lymphocytic Leukemia
Acute Myeloid Leukemia

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15
Q

Risk factor for leukemia

A
  • Radiation
  • Chemical exposure (formaldehyde and benzene)
  • Chemotherapy
  • Family hx
  • Down syndrome
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16
Q

Translocation involved in CML

A

Philadelphia chromosome: translocation between long arms of c22 and c9.

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17
Q

Sign and symptoms of AML and ALL

A
  • Sudden onset
  • Night sweats
  • Anemia
  • Fever
  • Bone pain
  • Bleeding and bruising
  • Potential CNS involvement: headaches, nausea, palsies
18
Q

Long term survival for adults with ALL or AML

A

30-40%

19
Q

Age group for CLL and CML

A

Primarily older with average age 72 yrs (CLL) and 67 yrs (CML) at diagnosis

20
Q

Hodgkin Lymphoma

A

cancers characterized by Reed-Sternberg cells that begins as a malignancy in a single lymph node and then spreads to contiguous lymph nodes

21
Q

Non Hodgkin Lymphoma

A

originate in the lymph nodes, are multicentric in origin
and spread early to various lymphoid tissues throughout the body, especially the liver, spleen, and bone marrow

22
Q

Signs and symptoms of CML and CLL

A
23
Q

Multiple myeloma

A

B cell malignancy of terminally differentiated plasma cells involving bone and bone marrow

24
Q

Why are people with multiple myeloma at risk for fractures and hypercalcemia?

A

It involves proliferation of osteoclasts, causing increased bone resorption and destruction

25
Q

What is hemostasis?

A

multistep physiological process that preserves vascular integrity by balancing the processes that maintain blood
in a fluid state and **prevent excessive bleeding **following injury

26
Q

What disorders would be associated with increased platelet numbers?

A

Reactive disorder (iron-deficiency anemia, splenectomy, cancer, chronic inflammatory conditions)
Myeloproliferative disorders (polycythemia vera)

27
Q

Lab value of thrombocytosis

A

elevations in the platelet count above 1,000,000/μL

28
Q

How do atherosclerotic plaques increase platelet function?

A

Atherosclerotic plaques disturb blood flow, causing endothelial damage and promoting platelet adherence.
Platelets adhere to the vessel wall, release growth factors, and cause proliferation of smooth muscle to contribute to the development of atherosclerosis

29
Q

What does thrombus formation result from?

A

Primary (genetic) or secondary (acquired) disorders that affect the coagulation components of the blood clotting cascade

30
Q

Common inherited factors causing hypercoagulability

A

Mutation in the factor V gene (factor V Leiden)
Mutation in the prothrombin gene

31
Q

What population is at risk for mutation in factor V gene?

A
  • Carried by 2-5% of white people
  • Mutation found in majority of people who have recurrent DVTs
  • One of the most common causes of primary and recurrent thromboembolism in pregnancy
  • associated with abruptio placentae (premature placental separation) and fetal growth disturbance
32
Q

What are conditions associated with acquired hypercoagulability?

A

Prolonged bed rest or immobility
Oral contraceptive agents and pregnancy
Myocardial infarction
Heart failure
Malignant diseases
Antiphospholipid antibody syndrome

33
Q

What is antiphospholipid syndrome?

A
  • autoantibodies directed against several negatively charged phospholipids, causing an increase in coagulation activity (mechanism unclear)
  • causes recurrent thrombosis, repeated fetal loss, and thrombocytopenia
  • seen with lupus
34
Q

What types of conditions are associated with arterial thrombi?

A

conditions that produce an increase in platelet numbers or turbulence in blood flow with platelet adhesion

35
Q

What conditions are associated with venous thrombi?

A

inherited or acquired conditions that cause a decrease in anticoagulation factors or
produce a stasis of blood, thereby causing an increase in procoagulation factors

36
Q

How does smoking cause thrombi?

A

increases sensitivity of platelets to factors that promote adhesiveness and aggregation

37
Q

Lab value of thrombocytopenia

A

decrease in the number of circulating platelets to a level less than 100,000/ μL

38
Q

Causes of thrombocytopenia

A

decreased platelet production
decreased platelet survival
splenic sequestration
dilution

39
Q

What conditions reduce platelet production?

A
  • leukemia
  • radiation and chemotherapy
  • anemia
40
Q

What causes reduced platelet survival

A

antiplatelet antibodies directed against platelet self-antigens or against antigens formed on platelets after blood transfusion or pregnancy

acute disseminated intravascular clotting or thrombotic thrombocytopenic purpura (TTP): platelets get used up with excessive clotting and leads to a deficiency.

41
Q

What is dilutional thrombocytopenia?

A

Massive blood or plasma transfusions may cause a dilutional thrombocytopenia because blood stored for more than 24 hours has no viable platelets

Pathophysiology (12 decks)

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