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ABBEY MSII Unit 2 > Rubin's 26. > Flashcards

Rubin's 26. Flashcards

1
Q

What is the origin of hematopoietic stem cells?

A

mesoderm

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2
Q

Where does blood cell formation take place in the fetus?

A

begins in yolk sac - moves to liver around month 3, go to bone marrow in 4th month

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3
Q

CFU-GEMM

A

Colony forming unit - multipotential cells: granulocyte, erythroid, macrophage, and megakaryocyte elements

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4
Q

CFU-L

A

colony forming unti lymphoid precursor cells

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5
Q

CFU-GM

A

granulocytic and monocytic cells

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6
Q

SCF

A

stem cell factor -support survival and proliferation of pluripotent stem cells, CFU-GEMM and various progenitor cells

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7
Q

Flt3 ligand (Flt3L)

A

support survival and proliferation of pluripotent stem cells, CFU-GEMM and various progenitor cells

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8
Q

Which cytokines support survival and proliferation of pluripotent stem cells, CFU-GEMM and various progenitor cells?

A

SCF and Flt3L

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9
Q

IL-3

A

important for proliferation of CFU-GEMM and multiple CFUs

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10
Q

GM-CSF

A

granulocyte-macrophage colony-stimulating factor: important for proliferation of CFU-GEMM and multiple CFUs

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11
Q

Which cytokines important for proliferation of CFU-GEMM and multiple CFUs?

A

IL-3 and GM-CSF

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12
Q

Erythropoietin

A

activates erythroid progenitor cells

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13
Q

Thrombopoietin (TPO)

A

facilitates production and maturation of megakaryocytic

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14
Q

How is a deficiency in one or more blood cell population treated?

A

growth factors: GM-CSF, G-CSF and EPO

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15
Q

What do progenitor cells mature into?

A

precursor cells called blasts

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16
Q

How do pro erythroblasts appear?

A

intensely basphilic cytoplasm with large round nucleus - maturation = progressive decrease of nucleus, increased nuclear chromatin density, progressive hemoglobinization of cytoplasm (changing it to red)

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17
Q

What defines erythroid lineage?

A

expression of glycophorin

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18
Q

How od myeloblasts appear?

A

round to oval nuclei with delicate chromatin, visible nucleoli, and blue-gray cytoplasm

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19
Q

How do promyelocytes appear?

A

similiar nuclei to myeloblasts (round to oval nuclei with delicate chromatin, visible nucleoli) but cytoplasm contains granules

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20
Q

How do mature neutrophils appear?

A

from promyelocytes: progressive nuclear chromatin condensation, increasing nuclear lobulation, appearance of secondary granules

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21
Q

How do megakaryocytic appear?

A

big multi lobed cells by endomitotic division

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22
Q

What is a common source of bone marrow for analysis in adults?

A

posterior iliac crest (rarely the sternum)

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23
Q

What is the fat-to-cell ratio in normal adult bone marrow?

A

50 to 50

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24
Q

What is the myeloid to erythroid ratio in normal adult bone marrow?

A

3:1 ish

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25
Q

Left shift

A

changes in the normal number and distribution of mature cells compared to immature cells

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26
Q

How can iron metabolism and storage be tested?

A

staining bone marrow aspirate with prussian blue: shows iron granules within cytoplasm of macrophages and nucleated red blood cell precursors

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27
Q

Where do RBCs get their color?

A

hemoglobin

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28
Q

What allows RBCs to deform their shape?

A

interconnected spectrin dimers and other stabilizing proteins (ankyrin, actin, band 4.1)

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29
Q

What is a cause of premature destruction of circulating RBCs?

A

changes in membrane-cytoskeletal unit that leads to increased cell rigidity

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30
Q

What is each hemoglobin molecule comprised of?

A

4 heme groups, 4 globin chains, which can transport 4 molecules of oxygen

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31
Q

What is the heme part of hemoglobin comprised of?

A

porphyrin ring (protoporphryin IX) and one ferrous iron ion

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32
Q

hemoglobin A

A

two alpha and two beta globin chains (normal)

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33
Q

Which types of hemoglobin are normally found in the blood in addition to hemoglobin A?

A

hemoglobin F (2 alpha and 2 gamma) and hemoglobin A2 (2 alpha and 2 delta)

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34
Q

Deoxygenated hemoglobin has a ______ oxygen affinity and requires _______ oxygen tension for heme-oxygen binding to occur

A

low; increased

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35
Q

Acidosis shifts the slop of the oxygen dissociation curve to the _______, which ________ tissue oxygen delivery.

A

right; increases

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36
Q

What is the average life span of blood erythrocyte?

A

120 dayss

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37
Q

What does the CBC measure?

A

hemoglobin, RBC count, and mean corpuscular volume

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38
Q

hematorcrit

A

MCV X RBC

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39
Q

RDW

A

variability in RBC size = RBC distribution width

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40
Q

poikilocytes

A

abnormally shaped RBCs

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41
Q

What are the four groups of anemia?

A
  1. acute blood loss; 2. decreased production by stem cell or progenitor cells; 3. ineffective hematopoiesis; 4. increased RBC destruction
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42
Q

What are the causes of microcytic anemias?

A

iron deficiency, thalassemias, sideroblastic

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43
Q

What are the causes of macrocytic anemias?

A

nutritional deficiency, alcohol, liver disease, hypothyroidism, reticulocytosis, primary bone marrow disease

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44
Q

What are the causes of normocytic anemia?

A

anemia of chronic disease/inflammation, anemia of renal disease, acute blood loss

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45
Q

What are the clinical features of anemia?

A

increased cardiac output; increased RR; shunting of blood flow to vital organs; decreased hgb-oxygen affinity; increased marrow erythrocyte production

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46
Q

What is the pathology of acute blood loss anemia?

A

initial - anemia not appreciated/noticed. within 24-48 hours, fluid is mobilized from extravascular to intravascular space to restore blood volume - red cell replacement not as rapid - will be gradually corrected

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47
Q

Iron deficiency

A

interfers with normal heme

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48
Q

hemosiderin

A

large aggregates of iron with a disorganized structure (as apposed to ferritin that is highly organized)

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49
Q

anisocytosis

A

variation in RBC size

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50
Q

What are lab findings on iron deficient anemia?

A

serum iron and ferritin low; total iron-binding capacity increased; transferrin saturation is lowered

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51
Q

What are the clinical features of advanced iron deficient anemia?

A

pica, smooth glistening tongue (atrophic glossitis), inflammation at corners of the mouth (angular stomatitis); koilonychia (spoon shaped deformity of fingernails)

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52
Q

Anemia of chronic disease

A

ineffective use of iron from macrophage stores in bone marrow

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53
Q

Which inflammatory cytokines inhibit iron mobilization?

A

lactoferrin, IL-1, TNF-a

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54
Q

Anemia of renal disease

A

decreased renal production of EPO leads to anemia: treat with recombinant EPO

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55
Q

How do erythrocytes appear in anemia of renal disease?

A

normocytic, normochromic, scalloped cell membranes (Burr cells) - can be fragmented/schistocytes if due to malignant hypertensions

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56
Q

Myelophthisic anemia

A

anemia associated with marro winfiltration: hypo proliferative anemia

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57
Q

What does the body do in an attempt to maintain blood cell production in myelophthisic anemia?

A

extramedullary hematopoisesis develops: mostly in spleen and liver

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58
Q

How do cells of myelophthisic anemia appear?

A

bone marrow infiltration causes moderate to severe normocytic anemia with anisopoikiocytosis and teardrop cells

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59
Q

Anemia of lead poisoning

A

lead interferes with several enzymes involved in heme synthesis

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60
Q

Megaloblastic anemia

A

impaired DNA synthesis because of vit B12 or folic acid deficiency - nuclear development delayed by cytoplasm matures normally

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61
Q

Which drugs can cause megaloblastic anemia?

A

chemotherapeutic agents (methotrxate, hydroxyurea) and antiretroviral drugs (5-azacytidine)

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62
Q

Where is folate absorbed?

A

jejunum

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63
Q

What lab value is elevated in folate and vitamin B12 deficiency?

A

Lactate dehydrogenase because of massive intramedullary destruction of red cell precursors

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64
Q

Schilling test

A

suggests the cause of B12 deficiency by measuring radio labeled vit B12 absorption, with or without intrinsic factor

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65
Q

How do folate deficiency and B12 deficiency differ clinically?

A

folate develops rapidly, B12 over years. Neuro symptoms with B12 (posterior and lateral column demyelination)

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66
Q

Thalassemia

A

congenital anemia caused by deficient global chain synthesis

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67
Q

Where is thalassemia most common?

A

Mediterranean and malaria endemic areas

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68
Q

How many alpha genes are there? Where are they located?

A

4 genes, each on chromosome 16 (4x4 = 16)

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69
Q

Beta thalassemia usually caused by what?

A

point mutation

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70
Q

Pathology of b-thalassemia

A

microcytic and hypochromic anemia - form unstable alpha tetramers that precipitate in the cytoplasm of developing erythroid precursors

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71
Q

What is characteristic of all forms of beta-thalassemia (how you’re able to tell the difference between alpha-thalassemia)

A

modest increase in HgbA2 (delta genes are upregulated)

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72
Q

Alpha thelassemia usually caused by what?

A

deletions

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73
Q

Heinz bodies

A

denatured hemoglobin - unstable hemoglobins H (B4) and Bart’s (gamma4) that precipitate in the cytoplasm

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74
Q

alpha thalassemia trait

A

two genes affected, mild microcytic anemia, up to 5% Hgb Bart

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75
Q

Mediterranean alpha thalassemia trait

A

single alpha gene deleted from each chromosome (SE asia, both genes from the same chromosome

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76
Q

Hemoglobin H disease

A

3 genes affected: moderate microcytic anemia: Hbg Bart up to 25% - Heinz bodies appear

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77
Q

Hemozygous alpha-thalassemia

A

all four genes affected: hydrous fettles, incompatible with life

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78
Q

What causes hemolytic anemia?

A

hemolysis (hehe)

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79
Q

Extravascular hemolysis

A

monocyte/macrophage system in spleen and a little liver is involved

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80
Q

intravascular hemolysis

A

RBCs destroyed while circulating

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81
Q

What are hemolytic anemias characterized by?

A

compensatory increase in red cell production and release

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82
Q

What are lab findings commonly associated with hemolysis?

A

increased LDH, increased bilirubin, decreased haptoglobin, free hemoglobin in blood and urine

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83
Q

How is the RBC membrane linked to the cytoskeleton?

A

spectrin (dimer of alpha and beta subunits); ankyrin (band 2.1) anchor spectrin to transmembrane proteins, and spectrin is bound to to actin and glycophorin by protein 4.1

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84
Q

hereditary spherocytosis

A

diverse group of inherited disorders of RBC cytoskeletons in which spectrin or another cytoskeletal component (ankyrin, protein 4.2, band 3) is deficient

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85
Q

What is a vertical defect?

A

deficiency of any cytoskeletal proteins (lipid bilayer uncoupled form the underlying cytoskeleton)

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86
Q

What does erythrocyte membrane defects lead to?

A

extravascular hemolysis

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87
Q

What is the genetics of hereditary spherocytosis?

A

AD

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88
Q

What is the pathology of hereditary spherocytosis (HS)?

A

normocytic anemia, hyperchromic cells, polychromes and reticulocytosis

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89
Q

Clinical features of HS

A

splenomegaly caused by chronic extravascular hemolysis - jaundiced, gallstones

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90
Q

When is transfusion needed with HS?

A

aplastic crisis (sudden decline in hemoglobin and reticulocites)

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91
Q

What usually causes aplastic crisis?

A

parvovirus B19

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92
Q

What is the risk with splenectomy

A

renders patient susceptible to infection - esp strep

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93
Q

Hereditary elliptocytosis (HE)

A

diverse group of inherited disorders affecting the erythrocyte cytoskeleton

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94
Q

What is the pathogenesis of HE (hereditary elliptocytosis)?

A

elliptical or oval RBCs due to defects in assemble of spectrum, spectrin-ankyrin binding, protein 4.1 and glycophorin C

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95
Q

Acanthocytosis

A

defect within the RBC membrane lipid bilayer and features irregularly spaced spiny projections of the surface - may be associated with hemolysis

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96
Q

Pathophysiology of acanthocytosis

A

MCC chronic liver disease - increased free cholesterol deposites in cell membranes

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97
Q

Pathology of acanthocytosis

A

irregular spiny surface projectiosn and central dense cytoplasm

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98
Q

Which enzyme defect can pre-dispose circulating RBCs to hemolysis?

A

glucose-6-phosphate dehydrogenase (G6PD) - catalyzes conversion of glucose-6-phosphate to 6-phosphogluconate - important because erythrocytes generate energy mainly by glycolysis

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99
Q

What are G6PD deficiency cause RBCs to be sensitive to?

A

oxidative stress (includes oxidant drugs such as the antimalarial primaquine)

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100
Q

What happens when G6PD RBCs are exposed to oxidative stress?

A

Heinz bodies appear with supra vital staining - bite cells appear because spleen bites parts off

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101
Q

What are most clinically relevant hemoglobinopathies caused by?

A

point mutations in the b-globin chain gene

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102
Q

Sickle cell disease

A

abnormal hemoglobin HgbS causes RBCs to sickle upon deoxygenation

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103
Q

How does HgbS heterozygosity protect against malaria?

A

infected erythrocytes selectively sickle and are removed from circulation by spleen and liver macrophages - DESTROY THE LITTLE SHIT

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104
Q

What is the molecular pathogenesis of sickle cell disease?

A

point mutation in the B-globin chain gene (valine for glutamic acid) which makes an unstable molecule that polymerizes like a bitch upon deoxygenation

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105
Q

What is the pathology of sickle cell disease?

A

severe normocytic or macrocytic anemia: howell-jolly bodies = nuclear remnants evident in most patients and reflect hyposplenism because of ischemic loss of splenic tissue

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106
Q

What happens in an aplastic crisis?

A

bone marrow fails to compensate for high level of red cell loss - hemoglobin level drop rapidly with no reticulocyte response (usually due to parvovirus B19)

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107
Q

Sequestration crisis

A

sudden pooling or erythrocytes esp in spleen decreases circulating blood volume and lowers hemoglobin levels

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108
Q

clinical manifestations of sickle cell disease

A

increased CO = cardiomegaly and CHF; acute chest syndrome; splenomegaly; neurologic issues vascular obstruction; retinal hemorrhage = blindness; increased bilirubin; cutaneous ulcers; handfoot syndrome in children

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109
Q

Why don’t sickle cell trait sickle?

A

HgbA in their RBCs prevents HgbS polymerization

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110
Q

What causes a milder form of sickle cell disease?

A

double heterozygosity for HgbS and HgbC - often develop retinopathy though

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111
Q

Hemoglobin C disease

A

homozygous inheritance of a structurally abnormal hgb, which increases erythrocyte rigidity and causes mild chronic hemolysis

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112
Q

Pathology of HgbC homozygosity

A

mild normocytic anemia

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113
Q

Hemoglobin E disease

A

homozygosity for a structurally abnormal hemoglobin, leading to a thalassemia-like defect that is associated with mild chronic hemolysis (unstable B-globin mRNA)

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114
Q

Warm antibody autoimmune hemolytic anemia

A

warm autoantibodies optimally bind their antigens at 37; IgG directed against erythrocyte membrane antigens (ie Rh group proteins) - RBCs are removed by macrophages of the reticuloendothelial system

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115
Q

Hapten mechanism Warm antibody autoimmune hemolytic anemia

A

drug such as penicillin attaches to RBC surface - elicited antibodies that react with erythrocyte itself

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116
Q

immune complex mechanism Warm antibody autoimmune hemolytic anemia

A

drug (quinidine) reacts with specific circulating antibody to form immune complexes which then bind to RBC membranes

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117
Q

Autoantibody mechanism Warm antibody autoimmune hemolytic anemia

A

drug (alpha-methyldopa) elicits antibodies (hemolysis occurs in the ABSENCE of the initiating drug)

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118
Q

Warm antibody autoimmune hemolytic anemia pathology and clinical features

A

normocytic or occasionally microcytic anemia - spherocytes and polychromasia

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119
Q

Direct Coombs test

A

helps to distinguish immune from nominee spherocytosis - patients RBCs are incubated with antihuman Ig - agglutination indicate antibody is present on cell surface

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120
Q

Cold antibody autoimmune hemolytic anemia

A

maximal reactivity at 4C due to mostly infections - so peripheral

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121
Q

What are cold antibodies? What are they directed against?

A

IgMs against I/i antigens on red cells

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122
Q

What are labs for cold agglutinins?

A

falsely low RBC counts and hematocrit, falsely elevated MCV and MCHC - direct Coombs test positive

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123
Q

What is the pathophysiology of cold hemolysin disease?

A

biphasic IgGs directed against P antigens on red cells - antibody binds erythrocytes at low temp and fixes compliment - intravascular hemolysis occurs when it warms back up

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124
Q

When does paroxysmal cold hemoglobinuria (PCH) - also called cold hemolysin disease - occur?

A

most often after viral illness

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125
Q

Lab values for paroxysmal cold hemoglobinuria/cold hemolysin disease

A

anemia, decreased haptoglobin, and hemoglobinuria due to intravascular hemolysis - Coombs test positive for complement

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126
Q

Hemolytic disease of the newborn

A

incompatibility of blood types between a mother and her developing fetus - mother lacks antigen present on fetal RBCs - IgG can cross placenta (usually Rh negative mother and Rh positive fetus)

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127
Q

What is characteristic of DIC?

A

abnormalities in coagulation and thrombocytopenia

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128
Q

What is characteristic of TTP?

A

thrombocytopenia alone

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129
Q

How does hypersplenism cause anemia?

A

spelomegaly causes pooling of blood and delayed transit of blood and delayed transit of blood cells through the splenic circulation and then prolonged exposure leads to premature destruction by macrophages

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130
Q

How do thermal burns cause anemia?

A

intravascular RBC hemolysis - membranes are disrupted and fragmented at temperatures over 49 degrees

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131
Q

Which infectious organisms cause RBC lysis?

A

plasmodium and babesiosis

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132
Q

polycythemia

A

an increase in RBC mass - when hematocrit is greater than 54% in men and 47% in women - this causes exponential increase in blood viscosity

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133
Q

relative polycythemia

A

occurs in dehydration - plasma volume is decreased - red cell mass is normal

134
Q

primary polycythemia

A

autonomous, EPO-independent proliferation of erythroid cells caused by an acquired, clonal, HSC disorder

135
Q

secondary polycythemia

A

EPO stimulation of erythropoiesis to compensate for general tissue hypoxia

136
Q

Normal platelet count

A

150-350X10^3

137
Q

TPO: what is it and where it is produced

A

stimulates thrombopoisesis and produced by the liver

138
Q

What do dense granules contain?

A

ADP (potent aggregating molecule) - also ATP, calcium, histamine, serotonine, epi

139
Q

What do alpha granules express on their membranes? What do they contain

A

adhesive proteins P-selectin on their membranes and contain fibrinogen, vWF, fibronectin and thrombospondin - also chemokine platelet factor 4, neutrophil0activating peptide 2, PDGF, TGF-a

140
Q

What does collagen bind?

A

GpIa/IIa and GpVI

141
Q

What does vWF bind?

A

Gp Ib/IX

142
Q

How does aggregation of platelets occur?

A

fibrinogen receptor Gp IIb/IIIa crosslinking

143
Q

thromboxane A2

A

provides positive feedback to activate additional platelets via their surface receptors

144
Q

Which complex initiates coagulation?

A

tissue factor (TF) and factor VIIa

145
Q

What does thrombin activate?

A

factors XI, VII, and V

146
Q

What activates protein C? What does protein C do?

A

anticoagulant complex of alpha thrombin-thrombomodulin - protein C inactivates VIIIa and Va

147
Q

antithrombin III

A

inhibits thrombin activity - also cleaves activated factors IXa, Xa, XIa, XIIa

148
Q

Plasminogen activators

A

produced by endothelial cells near the thrombus to activate circulating plasminogen to plasmin and initiate thrombolyysis

149
Q

What are the two plasminogen activators?

A

tissue plasminogen activator and urokinase-type plasminogen activator

150
Q

What do platelet abnormalities results in clinically?

A

petechiae and purpuric hemorrhages in skin and mucous membranes

151
Q

Senile purpura, purpura simplex, scurvy

A

senile: age-related atrophy of supporting connective tissues. purpura simplex: women during menses - deep dermis and resolves quickly. scurvy: vit c deficiency impairs collagen synthesis

152
Q

Hereditary hemorrhagic telangiectasia

A

autosomal dominant disorder of blood vessel walls (venules and capillaries) in which arteriovenous malformation and telangiectasis (dilated, tortuous small blood vessels) form in solid organs, mucous membranes, and dermis

153
Q

What is the underlying defect in HHT (hereditary hemorrhagic telangiectasia)

A

dilation and thinning of vessel walls due to inadequate elastic tissue and smooth muscle - mutations in ENG or ALK1

154
Q

Clinical features HHT (telangiectasias)

A

punctate reddish spots on the lips and nose, recurrent hemorrhages, anemic

155
Q

Henoch-Schonlein purpura

A

allergic purpura - vascular disease that results from immunologic damage to blood vessel walls (viral infections in children and drugs/chronic in adults)

156
Q

What is Henoch-Schonlein purpura characterized by?

A

leukocytoclastic vasculitis - perivascular iniltration of neutrophils and eosinophils, fibrinoid necrosis of vessel walls and platelet plugs in vascular lumens - IgA and complement complexes circulate in the blood and deposit in vessel walls

157
Q

Dhole-Like bodies

A

slightly abnormal neutrophils morphologically with blue cytoplasmic inclusions

158
Q

thrombocytopenia

A

platelet counts under 150,000

159
Q

Heparin-induced thrombocytopenia (HIT) type II

A

HEPARIN IS A HAPTEN immunologically mediated, caused by acquired IgG antibodies against platelet factor 4-heparin complexes - severe consumptive thrombocytopenia, platelet activation and thus a hyper coagulable state

160
Q

Heparin-induced thrombocytopenia (HIT) type I

A

HEPARIN ITSELF induces platelets to aggregate

161
Q

Glanzmann thrombasthenia

A

AR - defect in platelet aggregation caused by quantitative or qualitative abnormality in the gylcoprotein complex IIb/IIIa

162
Q

What is the most frequent complication of hemophilia A?

A

degenerative joint disease caused by repeated bleeding into many joints

163
Q

Factor IX

A

vitamin K dependent protein produced in the liver

164
Q

What three things does vWF bind?

A

platelet glycoprotein receptors (Gp Ib/IX), Gp IIb/IIIa for platelet aggregation, and factor VIII (in plasma for protection)

165
Q

Type I vWD (vWF disease)

A

AD: quantitative deficiency in vWF - all multimers are reduced

166
Q

Type II vWD

A

qualitative defects in vWF - interations of vWF and blood vessel wall defective

167
Q

Type III vWD

A

AR - vWF activity is absented plasma factor VIII levels are less than 10% of normol

168
Q

How does liver disease effect coagulation?

A

coagulation factors are produced by liver (II, V, VII, IX, X) - also has a key role in vit K absorption. PT, PTT prolonged (PT more affected)

169
Q

Vitamin K

A

essential cofactor in gamma carboxylation of glutamic acid residues - activating II, VII, IX, and X

170
Q

What are acquired inhibitors of coagulation factors?

A

circulating anticoagulants - IgG autoantibodies - usually against factor VII and vWF

171
Q

Factor V Leiden

A

mutation in factor V renders it resistant to inhibition to APC (activated protein C)

172
Q

Mild neutropenia

A

number of neutrophils is adequate to defend against microorganisms

173
Q

moderate neutropenia

A

patients become vulnerable to microbial infections (severe risk is high)

174
Q

What are the principal causes of neutropenia?

A

decreased production: irradiation, drug induced (including alcohol), viral infections, congenital, cyclic. ineffective production: megaloblastic anemia, myelodysplastic syndromes. increased destruction: isoimmune neonatal and autoimmune

175
Q

What drugs lead to immune0mediated neutrophil destruction?

A

sulfonamides, phenylbutazone, indomethacin

176
Q

What are clues to a benign (or reactive) nature of leukemia reaction (which can otherwise be mistaken for leukemia - CML)

A
  1. segmented neutrophils and fewer neutrophilic myeloid precursors 2. leukocyte alkaline phosphatase activity is HIGH (low in CML) 3. WBC under 50,000 4. reactive neutrophils often contain Dohle bodies (large blue cytoplasmic inclusions)
177
Q

Neutrophilia absolute neutrophil count

A

above 7000

178
Q

Eosinophil growth factor

A

IL-5

179
Q

Where do eosinophils hang out?

A

migrate from blood to gut, respiratory tract, and skin (where allergic reactions happen)

180
Q

When does hypereosinophilia happen?

A

mast cell disease, tumors (hodgkin and non-hodgkin lymphoma), myeloproliferative disorders

181
Q

What happens in hypereosinophilia?

A

accumulation of eosinophils in tissues leads to necrosis, particularly in the myocardium - grave prognosis - need aggressive corticosteroid therapy

182
Q

What do basophils do when stimulated?

A

synthesize leukotrienes and other mediators

183
Q

When does basophilia occur?

A

in immediate-type hypersensitivity reactions and with chronic myeloproliferative neoplasms

184
Q

What does monocytosis characterize?

A

both malignant and inflammatory conditions

185
Q

What are the symptoms of mast cell proliferative disease?

A

release of mast cell granules (inflammatory mediators histamine, heparin, eosinophil and neutrophil chemotactic factors, proteases) - causes flushing, pruritus, hives

186
Q

Aplastic anemia

A

disorder of pluripotential hematopoietic stem cells that leads to bone marrow failure - marrow is hypocellular and all blood cell lineages are decreased -> bad prognosis

187
Q

What causes aplastic anemia?

A

injury to hemtopoietic stem cells

188
Q

Which cell lineages are decreased in aplastic anemia?

A

myeloid, erythroid, and megakaryocytic - relative increase in marrow lymphocytes and plasma cells

189
Q

How does bone marrow appear on pathology in aplastic anemia?

A

large increase in fat cells

190
Q

What is the most common hereditary bone marrow failure syndrome? What is the associated gene?

A

Fanconi anemia: FANC gene (subtypes A, C, G, D2) - usually repair DNA

191
Q

Which cell lineages are affected by Fanconi anemia?

A

all hematopoietic cells

192
Q

What is the difference between Fanconi anemia and idiopathic aplastic anemia?

A

fanconi patients will not respond to immunosuppressive treatments (like they will with idiopathic aplastic anemia)

193
Q

What is the treatment of choice for Fanconi anemia?

A

HSC transplantation

194
Q

Pure Red Cell aplasia?

A

selective marrow suppression of committed erythroid precursors (unknown etiology)

195
Q

What is pure red cell aplasia most often due to?

A

viral infection (parvovirus B19) - acute or thymic lesions - chronic

196
Q

What are the receptors for parvovirus on red cell membranes?

A

P antigens

197
Q

What is the pathology of pure red cell aplasia?

A

overall marrow cellularity is normal, but erythroid precursors are absent or arrested in blast phase - increased EPO

198
Q

Paroxysmal Nocturnal hemoglobinuria

A

an acquired clonal stem cell disorder characterized by episodic intravascular hemolytic anemia due to increased RBC sensitivity to complement-mediated lysis

199
Q

What gene mutation causes paroxysmal nocturnal hemoglobinuria?

A

PIG-A (on X chromosome) - disrupts synthesis of GPI which anchors many proteins to RBC membranes

200
Q

What is the pathogenesis of paroxysmal nocturnal hemoglobinuria?

A

PIG-A mutation disrupts GPI synthesis which anchors many proteins to RBC membranes - consequent loss makes RBCs more susceptible to lysis by complement

201
Q

Myeloproliferative neoplasma

A

clonal stem cell disorders - increased proliferation of one or more myeloid lineages (grnaulocytes, erythrocytes, megakaryocytes, or mast cells)

202
Q

What are character features of all subtypes of myeloproliferative neoplasms?

A

bone marrow hypercellularity with effective hematopoietic maturation and increased numbers of red cells, granulocytes and/or platelets - also typically have bone marrow fibrosis and splenomegaly

203
Q

CML

A

chronic myelongenous leukemia - philadelphia chromosome BCR/ABL fusion gene

204
Q

What is the BCR/ABL fusion gene?

A

philly chromosome seen in CML - active tyrosine kinase central to pathogenesis of CML - autophosphorylates and activates downstream signaling pathways that trigger cell proliferation, differentiation, survival and adhesion

205
Q

CML chronic phase

A

leukocytosis consisting of neutrophils in all stages of maturation with a peak of mylocytes and mature neutrophils

206
Q

CML accelerated phase

A

disease progression with persistent or increasing WBC count, splenomegal, thrombocytopenia, additional chromosomal abnormalities, 20% blood basophila, and/or 10-19% blasts that are unresponsive to treatment

207
Q

CML blast phase

A

evloution to acute leukemia: 20% or more blasts, extra medullary proliferation, clusters of blasts in bone marrow. poor prognosis

208
Q

What is treatment for CML?

A

imatinib - tyrosine kinase inhibitor

209
Q

polycythemia vera

A

myeloproliferative neoplasm arising from a clonal HSC and characterized by autonomous production of RBCs, not responsive to EPO

210
Q

What are the criteria for polycythemia vera?

A

increased RBC mass, JAK2 mutation (no EPO elevation and hypercellular marrow - minor criteria)

211
Q

Primary myelofibrosis

A

clonal myeloproliferative neoplasm in which prominent megakaryopoisesis and granulopoisesis accompany marrow fibrosis - extra medullary hematopoiesis present in fully developed disease

212
Q

Essential thrombocythemia

A

megakaryocytes proliferate without resonant - blood platelet counts increased

213
Q

How does the pathology of essential thrombocythemia appear?

A

increased numbers of large, hyperlobulated, “stag-horn-shaped” megakaryocytic with abundant mature cytoplasm form cohesive clusters or sheets in the marrow

214
Q

Mastocytosis

A

clonal hematopoietic disorder in which neoplastic mast cells accumulate in certain tissues - mainly skin and bone marrow - depletion of fat and normal hematopoietic elements in marrow

215
Q

How does mastocytosis appear?

A

cutaneous: single or multiple lesions - tan-brown, cutaneous nodule in newborns

216
Q

How does the spleen appear in mastocytosis?

A

nodular aggregates of mast cells with accompanying dense fibrosis in both red and white pulp

217
Q

What are the clinical features of mastocytosis?

A

anaphylactic episodes - pruritus, flushing, hypotension, asthmatic symptoms and secondary anemia

218
Q

Myelodysplastic syndromes

A

peripheral blood cytopenias accompany a hypercellular marrow with ineffective hematopoiesis -

219
Q

What does myelodysplastic syndrome progress to 30-40% of the time?

A

AML

220
Q

AML diagnosis

A

more than 20% myeloblasts in the blood or bone marrow

221
Q

Acute promyelocytic leukemia

A

t(15;17)

222
Q

What is AML considered if there is less than 20% blasts?

A

MDS (myelodysplastic syndrome) or MPN (myeloproliferative neoplasm) category

223
Q

Auer Rods - what are they and what are they a sign of?

A

coalesced primary granules: appear eosinophilic, slender cytoplasmic inclusion. specific for myeloid lineage and preclude a diagnosis of lymphoblastic leukemia

224
Q

What is cytochemical stained to classify AML cases

A

Auer rods, myeloperoxidase, Sudan black, nonspecific esterase (NSE)

225
Q

Clinical features of AML?

A

most occur in adults - leukopenia, thrombocytopenia, anemia (progressive accumulation in the marrow of immature myeloid cells that cannot differentiate and mature further)

226
Q

Gene fusion in APL

A

PML/RARA

227
Q

How do patients with APL present?

A

DIC because senescent leukemic cells degranulate and activate the coagulation cascade

228
Q

How is APL treated?

A

with ATRA - induces maturation of the tumor cells and prevents both degranulation and DIC

229
Q

Myeloid sarcoma

A

extramedulllary solid tumor of myeloblasts and mono blasts

230
Q

MALT

A

mucosa-associated lymphoid tissue - aggregates of organized lymphoid tissue present in extra nodal sites including gut, lungs, skin

231
Q

What do Precursor B-cell acute lymphoblastic leukemias express

A

CD10 and CD19 - LACK CD20 (mature B cells) and Lchains

232
Q

What do plasma cells no longer express on their cell membranes?

A

CD20

233
Q

MCC reactive lymphocytosis

A

infectious mononucleosis due to EBV

234
Q

Bone marrow plasmacytosis greater than 10% typically associated with what?

A

plasma cell neoplasm

235
Q

Posterior auricular lymph nodes enlarged in….

A

rubella

236
Q

occipital lymph nodes enlarged in……

A

scalp infection

237
Q

posterior cervical lymph nodes enlarged in……

A

toxoplasmosis

238
Q

axillary lymph nodes enlarged in……

A

infections of the arms or chest wall

239
Q

inguinal lymph nodes enlarged in……

A

venereal infections and infections of the legs

240
Q

Follicular hyperplasia characteristic

A

starry sky pattern - scattered benign macrophages with abundant pale cytoplasm containing pyknotic nuclear and cytoplasmic debris

241
Q

Toxoplasmosis

A

prominent follicular hyperplasia and small collections of epithelioid macrophages in interfollicular regions and around the hyperplastic follicles (mixed pattern of reactive lymph node hyperplasia)

242
Q

Sinus histiocytosis

A

an increase in macrophages lining nodal sinuses - derive from blood monocytes

243
Q

Dermatopathic lymphadenopathy

A

lipid, melanin, and hemosiderin drain from affected skin to regional lymph nodes - T cell proliferation caused by chronic skin diseases

244
Q

MC childhood leukemia

A

precursor B-cell ALL

245
Q

Associated genetic/chromosomal abnormality b-lymphoblastic leukemia/lymphoma

A

t(9;22) translocation

246
Q

Associated genetic/chromosomal abnormality T-lymphoblastic leukemia/lymphoma

A

TCR genes translocate to sites involving MYC

247
Q

Associated genetic/chromosomal abnormality follicular lymphoma

A

t(14;18) - inactivation of p53; activation of MYC

248
Q

Associated genetic/chromosomal abnormality mantle cell lymphoma

A

t(11;14)

249
Q

Associated genetic/chromosomal abnormality marginal zone lymphoma

A

t(11;18)

250
Q

Associated genetic/chromosomal abnormality burkitt lymphoma

A

t(8;14) MYC

251
Q

Associated genetic/chromosomal abnormality plasma cell myeloma

A

clonal rearrangements involving Ig H and L genes - IgH translations with cyclin D

252
Q

What are clinical manifestations of precursor B-cell ALL leukemia?

A

proliferate in marrow and displace normal elements resulting in anemia, thrombocytopenia, and neutropenia - bone pain and arthralgia

253
Q

B-ALL prognosis

A

excellent yay

254
Q

What is the earliest T-cell antigen?

A

CD7

255
Q

Most frequent leukemic lymphoma

A

diffuse large B-cell lymphoma (followed by follicular)

256
Q

What favors development of large B-cell lymphoma or Burkitt lymphoma?

A

immunodeficiency

257
Q

Low grade B-cell lymphomas tend to develop in who? Burkitt lymphoma?

A

Bcell = autoimmune disease. Burkitt = EBC

258
Q

Marginal zone lymphomas

A

where late-stage memory B cells reside - outermost compartment of lymphoid follicles (which also give rise to CLL)

259
Q

What are examples of mature B-cell lymphomas?

A

B-cell CLL, follicular lymphoma, extra nodal marginal zone B-cell lymphoma (MALT), lymphoplasmacytic lymphoma

260
Q

With the B-cell lymphomas, which are curable and which are incurable?

A

curable = aggressive lymphomas that progress rapidly - curable with conventional therapies. non curable - indolent lymphomas that follow a prolonged clinical course

261
Q

B-cell CLL

A

common, mature CD5+ Bcell tumor - small lymphocytes - single basophilic nucleoli - CLL = only effects bone/bone marrow. lymphadenopathy or solid tumor called SLL

262
Q

Where does Bcell CLL/SLL infiltrate?

A

splenic white and red pulp and portal areas of the liver

263
Q

What are the clinical features of B-cell CLL/SLL?

A

asymptomatic - abnormal CBC with absolute lymphocytosis - immune deficiencies of B cells, some T cells

264
Q

Richeter syndrome - how does it appear

A

transformation from Bcell CLL to diffuse large b-cell lymphoma. appears as rapidly enlarging mass, worsening systemic symptoms, high lactate dehydrogenase level in serum

265
Q

Common complications of B-cel CLL/SLL

A

transformation to prolymphocytic leukemia (MC) and Richter syndrome (transformation to diffuse large B-cell lymphoma)

266
Q

Follicular lymphoma

A

mature-B-cell lymphoma of follicle center B cell (germinal center cells)

267
Q

Follicular cell genetic abnormality

A

14:18 = bcl-2 under control of IgH promoter - Bcl-2 overexpression (inhibitor of apoptosis usually)

268
Q

Clinical features of follicular lymphoma

A

generalized adenopathy - painless and may have waxing and waning course - this is incurable

269
Q

Mantle cell lymphoma

A

CD5+ mature B-cell tumors presenting a picture of monotonous small to medium sized lymphocytes

270
Q

Mantle cell lymphoma chromosome abnormality

A

11:14 - overexpression of cyclin D1 - cell cycle dysregulation now. incurable

271
Q

Characteristic feature of mantle cell lymphoma for pathology

A

striking monotony of lymphoma cells with respect to size and shape

272
Q

What cell marker are MCL cells positive for?

A

cyclin D1 (what helps to distinguish it from other lymphomas)

273
Q

Marginal zone lymphomas

A

mature B-cell tumor that arises in lymph nodes, spleen and extra nodal B cells (MALT) - memory B cells

274
Q

What is associated with a maltoma?

A

h. pylori gastritis - treatable with antibiotic therapy alone :0

275
Q

What distinguishes maltoma from B-CLL/SLL and mantle cell lymphoma?

A

negative for CD5, CD23, and cyclin D - most express IgM

276
Q

lymphoplasmacytic lymphoma

A

mature b-cell neoplasm containing small lymphocytes, plasmacytoi lymphocytes and plasma cells - involve bone marrow and sometimes spleen/lymph nodes

277
Q

What is the plasma cell marker of lymphoplasmacytic lymphoma? pathologic marker?

A

CD138 plasma cell - Dutcher bodies (immunoglobulin pseudo inclusions in the cell)

278
Q

Similarity between small B-cell lymphomas

A

incurable

279
Q

Hairy cell leukemia

A

clonal B-cell neoplasm small to medium sized - involve bone marrow and peripheral blood - arise from late activated memory B cell - have marked splenomegaly

280
Q

Hairy cell leukemia pathology

A

do not disturb the normal bone marrow architecture - have a “fried egg appearance” cell - spares lymph nodes but involves liver and spleen

281
Q

What do hair cell leukemia cells produce?

A

TRAP - tartrate-resistant acid phosphatase

282
Q

Diffuse large b-cell lymphoma

A

aggressive but curable b-cell tumor - most common - association with EBV

283
Q

Pathology of diffuse large b-cell lymphoma

A

lymphoma cells are twice the size of a normal lymphocyte - commonly presents at extra nodal sites esp GI tract

284
Q

How do diffuse large b-cell lymphoma patients present?

A

with a rapidly growing tumor in nodal and/or extranodal sites - symptoms reflect the site of involvement

285
Q

Burkitt lymphoma

A

most rapidly growing malignancy (so responds well to intensive chemotherapy)- chromosomal translocation involves MYC - presents at extra nodal site

286
Q

Who do B-cell lymphomas afflict?

A

more males than females

287
Q

How does sporadic Burkitt lymphoma present as compared to endemic burke lymphoma?

A

sporadic: abdominal mass involving ileocecum. endemic: EBV - involves jaw and facial bones

288
Q

How do cells appear under microscope for burkittt lymphoma?

A

macrophages ingesting cellular debris of apoptotic tumor cells = “starry sky” - have lipid vacuoles in deeply basophilic tumor cell cytoplasm

289
Q

Plasma cell neoplasia

A

clonal expansion of plasma cells

290
Q

Risk factors for plasma cell neoplasia

A

genetic predisposition, ionizing radiation, chronic antigenic stimulation

291
Q

Plasma cell myeloma

A

malignancy of plasma cells in which serum contains an M-protein - bone marrow based and multifocal (incurable still)

292
Q

Plasma cell myeloma pathology

A

destructive bone lesions - lytic or “punched out” appearance - show clusters, nodules, or sheets of plasma cells in bone marrow core

293
Q

High levels of M-protein in serum cause what?

A

erythrocyte rouleaux - RBC stick together end to end

294
Q

How can you tell the difference between a normal plasma cell and a myeloma cell?

A

myseloma cells lack CD19 and do not express CD20

295
Q

What is the heavy chain in plasma cell myeloma typically?

A

IgG or IgA (IgD and IgE are uncommon and aggressive - sometimes called plasma cell leukemia)

296
Q

Major risk factors for T-cell neoplasia

A

HTLV-1 and EBV

297
Q

How can you tell the difference between an immature T-cell neoplasm and a mature T-cel neoplasm?

A

mature T cell neoplasm lacks TdT

298
Q

Clinical features of peripheral T-cell and NK-cell tumors

A

more widely disseminated and more aggressive than B-cell neoplasms - respond poorly to chemo

299
Q

Adult T-cell leukemia/lymphoma

A

ATLL - caused by HTLV - Caribbean and Japan - long latency period

300
Q

ATLL pathology

A

usually widespread at presentation (involves lymph nodes, spleen, bone marrow, peripheral blood and skin) - have clonal T-cell receptor gene rearrangements and have clonal integrate HTLV-1

301
Q

Membrane markers ATLL

A

expresses CD25 and CD4 but absent CD7

302
Q

Clinical features ATLL

A

systemic with multiorgan manifestations, hypercalcemia, skin most important extra nodal site

303
Q

Mycosis Fengoides

A

most common form of primary cutaneous T-cell lymphoma - characterized by infiltration of epidermis by malignant CD4. 3 stages - eczematous stage, plaque stage, tumor stage

304
Q

Sezary syndrom

A

variant of MF - triad of erythroderma, generalized lymphadenopathy, circulating lymphoma cells in blood

305
Q

What distinguishes MF from inflammatory diseases?

A

clonal T-cell receptor gene rearrangements

306
Q

Anaplastic large cell lymphoma

A

mature T-cell tumors - CD30 lymphoid activation marker - nodal and extranodal sites (skin)

307
Q

Genetic abnormality anaplastic large cell lymphoma

A

translocation involving ALK gene = good prognosis. ALK negative ALCL = more aggressive

308
Q

Hallmark cells of anaplastic large cell lymphoma

A

irregular shaped nuclei, abundant cytoplasm with distinct eosinophilic area near the nucleus (also express CD30)

309
Q

Angioimmunoblastic T-cell lymphoma

A

aggressive peripheral T-cell lymphoma - neoplastic T-cell infiltrates expand the parametrical regions of lymph nodes and are associated with a striking proliferation of high endothelial venues

310
Q

What is present in nearly all cases of angioimmunoblastic t-cell lymphoma?

A

EBV even though it infects only B cells. idk

311
Q

Classical Hodgkin Lymphoma

A

inflammatory background and composed of mononuclear hodgkin cells and multinucleate reed-sternberg cells (owl’s eyes). the neoplastic cells in HL are derivatives of germinal center B cells

312
Q

What occurs in >98% of hodgkin/reed sternberg cells?

A

a clonal Ig gene rearrangement

313
Q

Clinical features hodgkin lymphoma

A

nontender peripheral adenopathy in a single lymph node or group of nodes (usually cervical and mediastinal)

314
Q

Posttransplant lymphoproliferative disorder

A

occur in people treated with immunosuppressive regimens because they have received solid organ, bone marrow or stem cell allografts

315
Q

What is the key risk factor for posttransplant lymphoproliferative disorder?

A

seronegativity for EBV at the time of transplantation (usually caused by EBV)

316
Q

Clinical features of PTLD (posttransplant lymphoproliferative disorder)

A

present with nonspecific symptoms (lethargy, malaise, weight loss, fever

317
Q

Hemophagocytic disorders

A

immunologic defect that results in immune dysregulation with consequent increases in certain cytokines leading to inadequately regulated T-cell and macrophage activation

318
Q

Criteria for diagnosis of hemophagocytic disorders

A
  1. fever 2. splenomegaly 3. anemia 4. thrombocytopenia 5. hypertriglyceridemia 6. hypofibrinogenemia
319
Q

Inherited hemophagocytic disorders vs acquired hemophagocytic disorders

A

inherited: PFR1 gene in children. acquired: viral infections (primary infections with EBV, CMV, HIV, parvovirus), malaria, E.coli, histoplasmosis

320
Q

T cell domain of spleen. B cell domain of spleen

A

T cell - periarteriolar lymphoid sheath. B cell - follicles and a perifolicular marginal zone

321
Q

Howell-Jolly bodies

A

remnants of nuclear DNA

322
Q

What can be removed from splenic macrophages without destroying the erythrocytes?

A
  1. Howell-Jolly bodies 2. Heinz bodies 3. sideritic granules (iron)
323
Q

How does the spleen appear in sickle cell anemia?

A

modestly enlarged with thickened, fibrotic capsule which causes a “sugar-coated” appearance

324
Q

Thymoma

A

neoplasms of thyme epithelial - most are benign - encapsulated, firm and gray to yellow tumor divided into lobules

325
Q

What do thymomas have a coincidence with?

A

myasthenia gravis

326
Q

paraneoplastic syndromes

A

tumor-associated clinical manifestations that occur distant to the tumor and are caused by the secretion of tumor cell products such as hormones, cytokines, growth factors, and tumor antigens

327
Q

paraneoplastic pruritus

A

itching lasting longer than 6 weeks

328
Q

sweet syndrome

A

an acute febrile neutrophilic dermatosis characterized by abrupt onset of papular skin lesions, accompanied by systemic symptoms: fever, arthralgia, malaise, headache and myalgia

329
Q

When is sweet syndrome normally seen?

A

acute myeloid leukemia and myelodysplastic syndromes

330
Q

paraneoplastic pemphigus

A

mucocutaneous disease presenting with painful oral and mucosal ulcerations, with concomitant blistering skin lesions

ABBEY MSII Unit 2 (19 decks)

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