Blood Disorders - Final Exam Flashcards

1
Q

Fluid of blood

A

Plasma

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2
Q

Cells of blood

A

WBC (leukocytes)
RBC (erythrocytes)
Platelets (thrombocytes)

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3
Q

Sites of hematopoiesis

A

Adults: calvarium, vertebrae, ribs, sternum, pelvis
Children: most bones
Fetus: most ones
Embryo: yolk sac, liver cords

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4
Q

Centrifuging of blood

A

Plasma at top
Buffy coat (WBC and platelets) in middle
RBC at bottom

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5
Q

Erythrocytes

A

For transportation of oxygen from lungs into peripheral tissues
Live in circulation for 120 days - broken down in spleen

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6
Q

Hemoglobin

A

Complex molecules consisting of four heme groups (Fe in middle) and four globins
97% Hemoglobin A

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7
Q

Fetal hemoglobin

A

Alpha chains and gamma chains

In children, traces in adults

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8
Q

Bilirubin

A

Heme is broken down and Fe is removed

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9
Q

Hemoglobin synthesis

A

Requires iron, Vit B12, Vit B6, folic acid

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10
Q

Erythropoietin

A

Kidney sensing O2 is low and secrete hormone to tell bone marrow to make more RBC

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11
Q

Thrombocytes

A

Platelets

Essential clotting factors

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12
Q

Malignant transformation of hematopoietic cells (2)

A

Solid tumors or leukemia

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13
Q

Anemia (3)

A

Reduction of Hb in blood - below normal levels
Less than reference range established in given population
Associated with appearance of abnormal Hb, reduction in RBC, structural abnormalities of RBC

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14
Q

Causes of anemia (3)

A
  1. Decreased hematopoiesis
  2. Abnormal hematopoiesis
  3. Increased loss or destruction of RBC
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15
Q

Decreased hematopoiesis (2)

A
  1. Bone marrow failure (aplastic anemia, myelophthisic anemia)
  2. Deficiencies of nutrients (vit B12, folic acid, protein)
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16
Q

Abnormal hematopoiesis

A

Usually consequence of genetic abnormalities

Sickle cell anemia

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17
Q

Increased loss and destruction of RBC (4)

A
  1. Bleeding
  2. Intrasplenic sequestration
  3. Immune hemolysis
  4. Infections
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18
Q

Aplastic anemia

symptoms: 5

A

Idiopathic, secondary
Bone marrow depletion of hematopoietic cells (consists only of fibroblasts, fat cells, scattered lymphocytes)
Anemia, leukopenia, thrombocytopenis
Uncontrollable infections, bleeding tendency, chronic fatigue, sleepiness, weakness

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19
Q

Iron deficiency anemia

(histology: 2
causes: 3)

A

Most common form
Hypochromic (pale), microcytic (small) RBC
Increased loss of iron, inadequate iron intake or absorption, increased iron requirements

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20
Q

Megablastic anemia

histology: 3

A

Caused by deficiency of vit B12 or folic acid
Large RBC (macrocytic anemia)
Bone marrow is hypercellular with numerous megaloblasts (abnormal RBC precursors)
Hypersegmentation of neutrophils

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21
Q

Vit B12 deficiency (3)

A
  1. Pernicious anemia
  2. Lack of gastric intrinsic factor
  3. Atrophic gastritis
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22
Q

Folic acid deficiency

A

Inadequate intake

Due to diet or malabsorption

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23
Q

Hemolytic anemia (4)

A
  1. Increased RBC destruction
  2. Intracorpuscular or extracorpuscular defects
  3. Compensatory erythroid hyperplasia of BM
  4. Hyperbilirubinemia (jaundice)
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24
Q

Intracorpuscular defects (3)

A
  1. Structural abnormalities
  2. Sickle cell anemia, thalassemia, hereditary spherocytosis
  3. Infection
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25
Q

Extracorpuscular defects

A
  1. Antibodies, infectious agents, mechanical factors
  2. Autoimmune hemolytic anemia, hemolytic disease of newborn, transfusion reactions, hemolytic anemia due to cardiac valve prosthesis, disseminated intravascular coagulation
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26
Q

Sickle cell anemia

A
Substitution of glutamic acid by valine
Synthesis of abnormal beta chain of globin
Most prevalent among African populations
Multiple infarctions in various organs
Jaundice
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27
Q

Sickle cell anemia - low oxygen

A

Hemoglobin S polymerizes and forms chain that deforms cells
Reversible if O2 is back up, quickly
All symptoms occur due to clotting

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28
Q

Long term complications of sickle cell anemia (3)

A
  1. Delayed intellectual development, neurologic deficits
    Largely avoided due to medical surveillance and appropriate management or crises
  2. Cardiopulmonary insufficiency
  3. Recurrent infections
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29
Q

Thalassemia

(histology: 1
symptoms: 5
developmental: 2)

A

Genetic defect in synthesis of HbA, do not produce enough
Reduced rate of globin chain synthesis
Beta or alpha chain affected
Minor (heterozygotes) or major (homozygotes, very serious)
Hypochromic (pale RBC) anemia
Splengomegali, hemosiderosis, hepatomegaly
Bone marrow: compensatory hyperplasia
Calvarium (crew cut) hair on radiographic study - a lot of hematopoiesis in skull and changes the way it looks
Jaundice
Chronic anemia that slows growth of children
Impaired normal intellectual development
Cardiorespiratory insufficiency

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30
Q

Immune hemolytic anemias

A

Mediated by antibodies that destroy RBC (autoantigens, alloantigens, neoantigens)
Mismatched blood transfusion
Hemolytic disease of new bown
Autoimmune hemolytic anemias

31
Q

Neoantigens

A

Caused by penicillin getting absorbed into RBC

32
Q

Polycythemia vera

A

Too much Hb
Primary + secondary
Erythrocytosis
Increased number of RBC

33
Q

Primary Polycythemia

A

Cancer
Myeloproliferative disorder
Clonal proliferation of hematopoietic stem cells
Uncontrolled production of RBC

34
Q

Secondary Polycythemia

A

Increased RBC volume due to erythroid bone marrow hyperplasia caused by erythropoietin
Usually caused by prolonged hypoxia

35
Q

Risk factors of secondary polycythemia (4)

A
  1. Living at high altitudes
  2. Anoxia secondary to chronic lung disease
  3. Congenital heart disease
  4. Renal carcinoma
36
Q

Polycythemia symptoms (7)

A
  1. Hypertension
  2. Dark red or flushed face
  3. Headaches
  4. Visual problems
  5. Neurologic symptoms
  6. Splegomegaly
  7. Hypercellular bone marrow
37
Q

Leukocytic disorders (4)

A
  1. Leukopenia
  2. Leukocytosis
  3. Leukemia
  4. Lymphoma
38
Q

Leukopenia

A

Reduction in WBC

Neutropenia or lymphopenia

39
Q

Neutropenia

A

Reduction in neutrophils

Caused by bacterial infections or drugs

40
Q

Lymphopenia

A

Reduction in lymphocytes

Bacteria, viral, fungal, parasitic infections

41
Q

Leukocytosis

A

Increased number of WBC in peripheral blood

Neutrophilia, eosinophilia, lymphocytosis, splengomegaly, lymphadenopathy

42
Q

Neutrophilia

A

Increased WBC

due to bacterial infection

43
Q

Eosinophilia

A

Increased WBC due to allergies, some skin diseases, parasitic infections

44
Q

Lymphocytosis

A

Increased WBC due to viral infections, chronic infections, some autoimmune disorders

45
Q

Malignant disease of WBC (3)

A
  1. Leukemias
  2. Lymphomas
  3. Plasma cell myeloma
46
Q

Leukemia

symptoms: 3

A

Malignant disease involving WBC or their precursors in BM, peripheral blood
Anemia, recurrent infections, uncontrollable bleeding
Myeloid or lymphoid

47
Q

Myeloid

A

From neutrophil, basophil, etc

48
Q

Lymphoid

A

B or T cells

49
Q

Lymphomas

locations: 5

A

Lymphoid cell malignant diseases predominantly involving lymph nodes
Most common in adults, aggressive forms in kids
Malignant cells infiltrate lymph nodes, spleen, thymus or BM or extranodal spread

50
Q

Non-Hodgkin’s lymphoma

A

B cell, T cell, NK cell
Most have B cell phenotype
All age groups (more common in adults)
Can spill over in blood and present as leukemia

51
Q

Hodgkin lymphoma

A
B cell only
Bimodal age distribution (25 +55)
5 types
Reed-Sternberg cells present
Enlarged lymph nodes
Stage I and II have excellent prognosis
Extranodal involvement and leukemic spread is rare
52
Q

Plasma cell myeloma

A

Malignant disease of plasma cells
High level of antibodies
Most patients older than 45
Malignant cells proliferate in BM

53
Q

HTLV-1

A

Causes lymphoma cancer

54
Q

EBV

A

Causes Burkitt’s lymphoma

55
Q

t(8,14)***

A

Burkitt’s lymphoma

56
Q

t(9,22)***

A

Chronic myelogenous leukemia

Philadelphia chromosome

57
Q

t(15,17)

A

Acute promyelocytic leukemia

58
Q

Acute lymphoblastic leukemia

treatment: 1

A

Immature form of cells
Very serious, can die immediately without treatment
Most common in children, almost always B cell origin
Massive infiltration of BM and peripheral blood with immature lymphoid cells (B cell blasts)
Modern chemotherapy

59
Q

Chronic myelogenous leukemia

treatment: 1

A

Mature type of cell
BM, peripheral blood overrunn with neutrophils and precursors
Three phases (chronic phase, accelerated phase, blast crisis)
Treatment with tyrosine kinase inhibitors
Philadelphia chromosome with BCR-ABL gene rearrangement (t9,22)

60
Q

Age distribution of leukemias

A

Rate goes up with age

except acute lymphocytic

61
Q

Acute myelogenous leukemia

A

Most common form of acute leukemia in adults
Clonal proliferation of myeloid precursors in BM (abnormal cells or cytogenic abnormalities)
Without treatment, most patients due within 6 months after symptom onset

62
Q

Chronic lymphocytic leukeumia

A

Malignant disease involving lymphocytes, mainly B cells
Most patients older than 50
Should be expected if lymphocytes are >5000/mL
Medial survival: 7-9 years after diagnosis

63
Q

Follicular lymphoma

symptoms: 2

A

Most common form of lymphoma in USA (45%)
Mostly seen in older people
Slow growing
Presents with long-standing elargement of lymphnodes, mild constitutional symptoms
Median survival: 7-9 years

64
Q

Diffuse large-cell lymphomas

A

Most aggressive form of NHL
Tissue infiltration by large lymphoid cells with irregular nuclear outlines, prominent nucleoli
Complete remission in 75% of patients with chemotherapy

65
Q

Burkitt’s Lymphoma

A

Highly malignant tumour composed of small B cells
Extranodal masses
Endemic or sporadic variants
Most children and young adults can be cured

66
Q

Endemic variant of Burkitt’s lymphoma

A

Sub-saharan Africa
Children infected with EBV
Mandible, facial soft tissue involvement

67
Q

Sporadic variant of Burkitt’s lymphoma

A

Children and young adults

Abdominal masses

68
Q

5 types of Hodgkin’s lymphoma

A
  1. Nodular sclerosing
  2. Lymphocyte predominant
  3. Lymphocyte rich
  4. Mixed cellularity
  5. Lymphocyte depleted
69
Q

Reed-Sternberg cells (2)

A

Bilobed or multilobed nucleus, prominent nucleoli surrounded by clear halo

70
Q

Multiple myeloma

A

Plasma cell myeloma

71
Q

Diagnosis of plasma cell myeloma (4)

A
  1. X ray studies
  2. Serum electrophoresis
  3. Bone marrow biopsy
  4. Clinical presentation
    Survival rate is less than 3-4 years from diagnosis
    Most die from kidney failure and infection
72
Q

Diagnosis of Non-Hodgkin’s lymphoma (2)

A
  1. Light microscopy

2. Ancillary techniques (flow cytometry, immunositochemistry, cytogenetic analysis)

73
Q

Clinical features of Non-Hodgkin’s lymphoma (3)

A
  1. Lymph node enlargement
  2. Systemic consitutional symptoms
  3. Extranodal tumor spread
74
Q

Symptoms of plasma cell myeloma (5)

A
  1. Lytic lesions (punched out holes) in calvaria vertebrae
  2. Hypercalcemia (osteoclast function)
  3. Renal failure
  4. Anemia
  5. Leukopenia