Blood Disorders Flashcards

1
Q

What cell types are found in the buffy coat?

A

White blood cells

Platelets

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2
Q

Hemoglobin

A

Complex molecule consisting of 4 heme groups and 4 globins
Always 2 alpha chains, adult has beta, fetus has gamma
Purpose is to transport oxygen
Need iron!
Gets broken down to bilirubin
Live in circulation for 120 days

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3
Q

What 4 things are needed for hemoglobin synthesis?

A

Iron
Vitamin B12
Vitamin B6
Folic acid

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4
Q

How is hemoglobin degraded?

A

Split into heme and globin
Globin is degraded further into amino acids which are reutilized
Heme is broken into iron and bilirubin (iron reused, bilirubin to liver and turned into bile)

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5
Q

What is anemia?

A

Reduction of hemoglobin in blood to below normal levels
May be associated with appearance of abnormal hemoglobin, reduced number of RBCs, or structural abnormalities of RBCs
Decreased hematopoiesis, abnormal hematopoiesis, or increased loss/destruction of RBCs

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6
Q

2 causes of decreased hematopoiesis

A

Bone marrow failure (aplastic anemia or myelophthisic anemia)
Deficiencies of nutrients (low B12 and folic acid, or protein deficiency)

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7
Q

Myelophthistic anemia

A

Bone marrow stem cells are damaged or replaced by infiltrates of metastatic tumors cells

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8
Q

Cause of abnormal hematopoiesis

A

Usually a consequence of genetic abnormalities

Ex: sickle cell anemia

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9
Q

4 causes of anemia from increased loss and destruction of RBCs

A

Bleeding
Intrasplenic sequestration
Immune hemolysis
Infections (malaria)

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10
Q

Aplastic anemia

A

Idiopathic, secondary
Bone marrow depleted of hematopoetic cells - only fibroblasts, fat cells, and scattered lymphocytes
Causes anemia, leukopenia, and thrombocytopenia
Uncontrollable infections, bleeding tendency, chronic fatigue, sleepiness, weakness
Stem cells disappear from the bone marrow with consequent pancytopenia (lack of all blood cells) in the periphery

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11
Q

Iron deficiency anemia

A

Most common form of anemia
Hypochromic, microcytic anemia
Etiology: increased loss of iron, inadequate iron intake or absorption, increased iron requirements

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12
Q
Megaloblastic anemia
(caused by, morphology, what are the deficiencies from, effects on bone marrow/peripheral blood/neutrophils)
A

Caused by deficiency of vitamin B12 or folic acid
Opposite morphology from iron deficiency anemia (large red cells)
Lack of B12 from: pernicious anemia, lack of GIF, atrophic gastritis
Lack of folic acid from: inadequate intake in diet or malabsorption caused by intestinal disease
Bone marrow gets hypercellular with numerous megaloblasts
Macrocytic anemia in peripheral blood
Hypersegmentation of neutrophils

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13
Q

Definitions

  1. Hypochromic
  2. Microcytic
  3. Macrocytic
A
  1. Pale red cells
  2. Small red cells
  3. Large red cells
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14
Q

Hemolytic Anemia

A

Increased red blood cell destruction (hemolysis)
Intra and extra cellular defects
Compensatory erythroid hyperplasia of bone marrow
Hyperbilirubinemia, jaundice

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15
Q

Intracorpuscular defects for hemolytic anemia

A

Structural abnormalities
Sickle cell anemia, thalassemia, hereditary spherocytosis
Infection (malaria)

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16
Q

Extracorpuscular defects for hemolytic anemia

A

Antibodies, infectious agents, mechanical factors
Autoimmune hemolytic anemia, hemolytic disease of the newborn, transfusion reactions, hemolytic anemia caused by cardiac valve prosthesis, DIC

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17
Q

Sickle cell anemia

A

Substitution of glutamic acid by valine
Synthesis of abnormal beta chain of globin
Multiple infarcts in various organs (neurologic defects, sharp pain in bones, spleen, extremities, retinal infarcts)
Hyperbilirubinemia, jaundice

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18
Q

Long term complications of sickle cell anemia

A

Delayed intellectual development, neurologic deficits (largely avoided)
Cardiopulmonary insufficiency
Recurrent infections

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19
Q

Thalassemia

A

Genetic defect in synthesis of hemoglobin A, reduced rate of globin chain synthesis
No abnormal hemoglobin produced
Beta and alpha types depending on what chain has reduced synthesis
Calvarium, hyperbilirubinemia, jaundice
Chronic anemia that slows children’s growth
Impairment of intellectual development
Cardiorespiratory insufficiency

20
Q

Thalassemia minor/trait vs thalassemia major

A

Minor: heterozygotes, mild non specific symptoms
Major: homozygotes, severe serious disease

21
Q

Calvarium

A

“Crew cut” hair on readiographic study
There is a lot of hematopoiesis happening in the skull which is abnormal
In thalassemia

22
Q

Definitions:

  1. Autoantigens
  2. Alloantigens
  3. Neoantigens
A
  1. Antibodies you make against yourself
  2. Against someone else’s RBCs
  3. When 2 things come together. Ex: penicillin gets absorbed into red cell membranes and forms something that causes antibodies to form
23
Q

Polycythemia (difference between primary and secondary)

A

Increased numbers of RBCs
Primary (polycythemia vera): clonal proliferation of hematopoietic stem cells resulting in an uncontrolled production of RBCs and an increased total red blood cell mass - erythropoietin INdependent
Secondary: increased RBC volume because of erythroid bone marrow hyperplasia caused by erythropoietin - caused by hypoxia

24
Q

Symptoms of Polycythemia

A
Hypertension
Dark red or flushed face
Headaches, visual problems, neurologic symptoms
Splenomegaly
Hypercellular bone marrow
25
Q

4 leukocytic disorders

A

Leukopenia
Leukocytosis
Leukemia
Lymphoma

26
Q

Leukopenia

A

Reduction in WBC count
Neutropenia due to bacterial infections or drugs
Lymphopenia due to bacterial, viral, fungal, or parasitic infections

27
Q

Leukocytosis

A

Increased number of WBCs in peripheral blood
Neutrophilia from bacterial infection
Eosinophilia: allergies, some skin diseases, or parasitic infections
Lymphocytosis: viral infections, chronic infections, some autoimmune disorders
Splenomegaly, lymphadenopathy

28
Q

Leukemia

A

Malignant disease involving WBCs or their precursors in bone marrow, peripheral blood (acute and chronic)
Can be myeloid or lymphoid
Bone marrow overrun with leukemia cells
Increased number of immature blood cells in peripheral blood
Complications: anemia, recurrent infections, uncontrollable bleeding
Doesn’t make red/white cells or platelets normally

29
Q

Lymphomas

A

Lymphoid cell malignant diseases predominantly involving lymph nodes
Non-Hodgkins or Hodgkins

30
Q

Plasma cell myeloma

A

Malignant disease of plasma cells

31
Q

Non-Hodgkin’s vs Hodgkin’s lymphoma

A

NH: B, T, or NK cells
H: B cell only

32
Q

Translocations:

  1. 8,14
  2. 9,22
  3. 15, 17
A
  1. Burkitt’s lymphoma
  2. Chronic myelogenous leukemia (Philadelphia chromosome)
  3. Acute promyelocytic leukemia
33
Q

Acute lymphoblastic leukemia

A

Most common form of leukemia in children
Almost always B cell origin
Massive infiltration of bone marrow, peripheral blood with immature lymphoid cells
Symptoms: recurrent infections, weakness, bleeding into skin and major internal organs
Treatment: chemo - remission possible in nearly all patients

34
Q

Acute Myelogenous Leukemia

A

Most common form of acute leukemia in adults
Clonal proliferation of myeloid precursors in bone marrow
20% abnormal cells, or cytogenetic abnormalities
Without treatment, most patients die within 6 months after symptom onset

35
Q

Chronic Myelogenous Leukemia

A

Bone marrow, peripheral blood overrun with neutrophils and their precursors
3 phases: chronic, accelerated, blast crisis
Treatment: tyrosine kinase inhibitors
Philadelphia chromosome, with BCR-ABL gene rearrangment t(9;22)

36
Q

Chronic lymphocytic leukemia

A

Malignant disease involving lymphocytes (almost all B cells)
Most patients over 50
Should be suspected if lymphocytes greater than 5000/mL
Median survival: 7-9 years from diagnosis

37
Q

Non-Hodgkin’s Lymphomas

A

No benign lymphomas
Most have B-cell phenotype
All age groups affected (more common in adults than children)
Can spill over into blood; present as leukemia
Involve lymph nodes, bone marrow, spleen, thymus, but can be of extranodal origin

38
Q

Follicular lymphoma

A

Most common form of lymphoma in US
Mostly older people
Slow growing
Presents with long-standing enlargement of lymph nodes, mild constitutional symptoms
Median survival: 7-9 years after diagnosis

39
Q

Diffuse Large-Cell Lymphomas

A

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

40
Q

Is it easier to treat fast or slow growing cancers? Why?

A

Fast growing

If they grow more rapidly they are more sensitive to the chemos

41
Q

Burkitt’s Lymphoma

A

Highly malignant tumor composed of small B-cells
Extranodal masses: more prominent than enlarged lymph nodes
Endemic and sporadic variant
Most children and young adults can be cured

42
Q

Hodgkin’s Lymphoma

A

Bimodal age distribution curve: peaks at 25 and 55 years
Five types
Reed-Sternberg cells present
Clinically: enlarged lymph nodes, rarely extranodal involvement or leukemic spread

43
Q

5 types of HL

A
Nodular sclerosing
Lymphocyte rich
Mixed cellularity
Lymphocyte depleted
Lymphocyte predominant (non classical)
44
Q

Classical vs Nonclassical HL

A

Reed-Sternberg cells present in classical only

45
Q

Reed-Sternberg cells

A

Bilobed or multilobed nucleus, prominent nucleoli surrounded by clear halo

46
Q

Plasma Cell Myeloma

A
Malignant disease of plasma cells
Most patients older than 45
Proliferate in bone marrow
Punched out holes in calvaria vertebrae
Hypercalcemia, renal failure, anemia, leukopenia