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Heme and Lymph > RBC 1 > Flashcards

RBC 1 Flashcards

1
Q

Size and color: generally disorders of hemoglobin synthesis, often iron deficiency

A

Microcytic normochromic

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2
Q

Size: generally from impaired maturation of red cell precursors in BM. B12 and folate deficiency. pernicious anemia

A

Macrocytic

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3
Q

size and color: anemia of chronic disease

A

normocytic normochromic

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4
Q

normocytic value

A

80-100

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5
Q

What measurements are useful for color of RBCs

A
  • mean cell hemoglobin

- mean cell hemoglobin concentration

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6
Q

what is normal red cell distribution width

A

12-15

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7
Q

Older male above 60 with iron deficiency anemia. what is the workup?
if it’s a female?

A

colon cancer

-may be perimenopausal or gynecological bleeding but could also be colon cancer

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8
Q

is the HCT clinically useful in evaluating acute blood loss?

A

No, you are losing both red cells and plasma

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9
Q

How quickly are reticulocytes produced

A

5 days

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10
Q

What are the features of hemolytic anemias?

A
  • shortened red cell life span below the typical 120 days
  • Elevated erythropoietin levels and a compensatory increase in erythropoiesis
  • Hemoglobin degradation products tend to accumulate during red cell hemolysis
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11
Q

Predominant features of extravascular hemolytic anemia

A
  • anemia
  • SPLENOMEGALY
  • and jaundice
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12
Q

features of intravascular hemolytic anemia?

A
  • anemia
  • hemoglobinemia
  • hemoglobinuria
  • hemosidinuria
  • Jaundice
  • NO SPLENOMEGALY
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13
Q

Role of haptoglobin in hemolytic anemia

A

it binds degradation product so it goes down

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14
Q

in all types of uncomplicated hemolytic anemias, the excess serum bilirubin is _____

A

unconjugated

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15
Q

an inherited disorder caused by intrinsic defects in the red cell membrane skeleton that render red cells spheroid, less deformable, and vulnerable to splenic sequestration and destruction

A

Hereditary spherocytosis (HS)

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16
Q

what is the inheritance pattern of 75% of hereditary spherocytosis

A

autosomal dominant

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17
Q

in 2/3 of HS, the red cells are abnormally sensitive to what?

A

osmotic lysis when incubated in hypotonic salt solutions which causes the influx of water into sperocytes with little margin for expansion

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18
Q

what is the Mean cell hemoglobin concentration (MCHC) in HS

A

increased due to dehydration caused by loss of K+ and water

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19
Q

What are the characteristic clinical features of HS?

A
  • anemia
  • splenomegaly
  • Jaundice
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20
Q

The generally stable course of HS is sometimes puncuated by aplastic crises, usually triggered by what?

A

an acute parvovirus infection

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21
Q

describe the hemolytic crisis that can occur in HS?

A

produced by intercurrent events leading to increased splenic destruction of red cells (infectious mononucleosis)

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22
Q

What stones are found in many HS pts?

A

gallstones

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23
Q

What treats the anemia of HS?

A

splenectomy but brings with it an increased risk of sepsis

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24
Q

The episodic hemolysis that is characteristic of G6PD deficiency is caused by exposures that generate oxidant stress . . what are most common causes?

A
  • infections
  • Antimalarial drugs (like quinidine)
  • Tonic water from gin and tonics
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25
Q

Heinz bodies

A

G6PD deficiency

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26
Q

Bite cells

A

G6PD deficiency

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27
Q

A common hereditary hemoglobinopathy caused by a point mutation in b-globin that promotes the polymerization of deoxygenated hemoglobin, leading to red cell distortion, hemolytic anemia, microvascular obstruction, and ischemic tissue damage

A

Sickle cell Disease

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28
Q

what is common in children with sickle cell disease

A

painful bone crisis that is difficult to distinguish from acute osteomyelitis

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29
Q

What is a particularly dangerous type of vaso-occlusive crisis involving the lungs in sickle cell disease

A

Acute chest syndrome which typically present with fever, cough, chest pain, and pulmonary infiltrates

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30
Q

In sickle cell disease, what affects up to 45% of males after puberty and may lead to hypoxic damage and erectile dysfunction

A

Priapism

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31
Q

other disorders related to vascular obstruction in sickle cell disease

A

-stroke and retinopathy leading to loss of visual acuity

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32
Q

What are the factors proposed to contribute to stroke in sickle cell disease

A

adhesion of sickled red cells to arterial vascular endothelium and vasocontriction caused by the depletion of NO by free hemoglobin

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33
Q

Aplastic crisis in sickle cell disease stem from infection of red cell progrenitors by what?

A

parvovirus B19

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34
Q

A heterogeneous group of disorders caused by inherited mutations that decrease the synthesis of either the alpha or beta globin chains that compose adult hemoglobin

A

Thalassemias

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35
Q

Beta thallasemias are mainly what type of genetic abnormality?
Alpha?

A

point mutations

gene deletions

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36
Q

What beta thalassemia requires blood transfusions?

Which is severe but doesn’t require blood transfusions?

Which is Asymptomatic with mild or absent anemia; Target cells

A

Major

intermedia

minor

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37
Q

genotype for an alpha thalassemia silent carrier?

trait?

HBH disease?

Hydrops fetalis?

A
  • /a a/a
  • /- a/a (asian) or -/a -/a (Black african, asian)
  • /- -/a
  • /- -/-
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38
Q

size and color anemia for B-Thalassemia

A

hypochromic, microcytic

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39
Q

Where is B-Thalassemia Major most common

A

Mediterranean countries, parts of Africa, Southeast Asia

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40
Q

When does B-Thalassemia Major manifest?

A

6 to 9 months after birth as hemoglobin synthesis switches from HbF to HbA

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41
Q

What is the Major red cell hemoglobin in B-thalassemia Major?
What are the levels of HbA2?

A

HbF is markedly elevated

sometimes high but more often are normal or low

42
Q

describe the cheek bones in B-Thalassemia Major pts if they survive long enough?
other clinical findings?

A

enlarged and distorted . . crew cut appearance on X ray

Hepatosplenomegaly due to extramedullary hematopoiesis

43
Q

Describe the complications of blood transfusions in a B-Thalassemia major patient

A

Cardiac disease resulting from progressive iron overload and secondary hemochromatosis is an important cause of death, . . must be treated with iron chelators like EDTA

44
Q

What is the only therapy offering a cure for B-Thalassemia Major

A

Hematopoietic stem cell transplantation

45
Q

Hemoglobin elecrophoresis in B-Thalassemia minor shows what?

A
  • an increase in HbA2

- HbF levels are generally normal or occasionally slightly increased

46
Q

Recognition of B-Thalassemia minor is important for what 2 reasons?

A
  • it superficially resembles the hypochromic microcytic anemia of iron deficiency
  • it has implications for genetic counseling

There are women who are pregnant and have b thalassemia minor undiagnosed and present with microcytic hypochromic anemia and it’s immediately thought that they have iron deficiency anemia . . . Hb electrophoresis is called for . . .THE INCREASE IN HbA2 is DIAGNOSTICALLY USEFUL

47
Q

in newborns with alpha-Thalassemia, excess unpaired gamma-globin chains form gamma4 tetramers known as what?
Which alpha thalassemias is this found in?

A

hemoglobin barts

Silent carrier and trait

48
Q

A disease that result from acquired mutations in the phosphatidylinositol glycan complementation group A gene (PIGA), an enzyme that is essential for the synthesis of certain membrane associated complement regulatory proteins

A

Paroxysmal nocturnal hemoglobinuria (PNH)

49
Q

what is the only hemolytic anemia caused by an acquired genetic defect?

A

PNH

50
Q

what is the leading cause of disease related death in individuals with PNH

A

Thrombosis: about 40% of patients suffer from venous thrombosis

51
Q

About 5 -10% of PNH pts eventually develop what?

A

AML or a myelodysplastic syndrome

52
Q

How is PNH diagnosed?

A

by flow cytometry . . red cells deficient in CD59

53
Q

hemolytic anemias in this category are caused by antibodies that bind to red cells, leading to their premature destruction

A

immunohemolytic anemia . . commonly called autoimmune hemolytic anemia

54
Q

What ig is the culprit in warm antibody type immunohymolytic anemia?
What are the causes?

A

IgG

  • Primary
  • Secondary (SLE), drugs,
55
Q

What ig is the culprit in cold agglutinin type immunohymolytic anemia?
What are the causes?

A

IgM

  • Acute: mycoplasm infection, infectious mononucleosis)
  • Chronic: lymphoid neoplasms
56
Q

What ig is the culprit in cold hemolysin type immunohymolytic anemia?
What are the causes?

A
  • IgG

- mainly in children following viral infections

57
Q

Patients red cells are mixed with sera containing antibodies that are specific for human Ig or complement

A

DIRECT Coombs tests

58
Q

Patients serum is tested for its ability to agglutinate commercially availble red cells bearing particular defined antigens

A

INDIRECT coombs test

59
Q

The most significant hemolysis caused by trauma to red cells is seen in what individuals?

A

those with cardiac valve prosthesis and microangiopathic disorders

60
Q

Microangiopathic hemolytic anemia is most commonly seen with what?

A

DIC

61
Q

microvascular lesion that results in luminal narrowing often due to the deposition of fibrin and platelets

A

microangiopathic hemolytic anemia

62
Q

Red cell fragments (schistocytes), burr cells, helmet cells, triangle cells

A

Traumatic damage

63
Q

The most common and important anemias associated with red cell underproduction are those caused by what?

A

nutritional deficiencies

64
Q

What dietary deficiencies cause megaloblastic anemia

A

Vitamin B12 and folic acid

65
Q

What is one of the major white cell effects of megaloblastic anemia?

A

-multi lobated PMNs or nutrophils with five or more nuclear lobules

66
Q

Neurologic complications of B12 deficiency

A

-spastic paraparesis, sensory ataxia and severe parathesias of the lower limbs

67
Q

a specific form of megaloblastic anemia caused by autoimmune gastritis that impairs production of IF, required for vitamin B12 uptake from GI

A

Pernicious anemia

68
Q

Age for pernicious anemia

A

median of 60. rare under 30

69
Q

A diagnosis of pernicious anemia is based on what?

A
  • moderate to severe megaloblastic anemia
  • Leukopenia with hypersegmented granulocytes
  • Low serum B12
  • Elevated serum levels of homocysteine and methymalonic acid
70
Q

in a true case of pernicious anemia, what happens about 5 days after parenteral administration of B12

A

reticulocytosis and a rising HCT

71
Q

What is often found in B12 deficiency that is NOT related to pernicious anemia

A

-Achlorhydria and loss of pepsin secretion, vitamin B12 is not readily released from protein in food

72
Q

list the things that can cause B12 deficiency that are NOT pernicious anemia

A
  • Gastrectomy
  • loss of exocrine pancreas function
  • Illeal resection
  • tapeworms from raw fish
  • increased demand (pregnancy, hyperthyroidism, disseminated cancer, chronic infection)
73
Q

What are the manifestations of folic acid deficiency?

A

exactly the same as B12 deficiency

74
Q

What foods are the source of folic acid?

B vitamins?

A

green veggies

Meats

75
Q

Why is it essential to exclude B12 deficiency in megaloblastic anemia before initiating folate therapy?

A

folate does not prevent and may even exacerbate the neurologic deficits seen in B12 deficiency states

76
Q

What is the Schilling test

A

its a way to tell the difference b/t pure B12 deficiency and pernicious anemia but uses radioactive substances

77
Q

pigments involved in the formation of heme?

A

porphyrins

78
Q

Clinical effects of chronic Porphyria?

A
  • seen in skin (abnormal reactions to sunlight, blistering, abnormal hair growth)
  • teeth (staining)
79
Q

clinical effects of acute Porphyria?

A

-nervous system (referred pain thorax and abdomen), seizures, hallucinations, general psychosis

80
Q

What is the most common nutritional disorder in the world

A

Iron deficiency anemia

81
Q

plasma ferritin levels in Iron deficiency anemia

A

<12

82
Q

High iron in med is considered a risk factor for what?

A

Atherosclerosis because it is related to chronic inflammation

83
Q

What molecule is used for iron absorption

A

hepcidin

84
Q

hepcidin level in anemia of chronic disease

A

Increased

85
Q

What is the most common cause of iron deficiency in the Western world

A

Chronic blood loss

86
Q

Iron deficiency anemia in men and post menopausal women in the western world should be ascribed to what until proven otherwise

A

Occult blood loss. . . GI

87
Q

Describe the size and color of RBCs at first with IDA

A

normocytic normochromic

88
Q

describe RBCs in well established IDA

A

microcytic hypochromic anemia with modest poikilocytosis

89
Q
  • Microcytic hypochromic anemia
  • Esophageal Webs
  • Atrophic glossitis
A

Plummer-Vinson syndrome

90
Q

Diagnosis of IDA

A
  • HCT, Hgb decreased
  • serum iron: low
  • serum ferritin: low
  • TIBC; Total Iron binding capacity: high
  • Serum hepcidin: low
91
Q

what is considered the most common type of anemia in hospitalized patients in the US?
3 major categories in this setting?

A

impaired red cell production associated with chronic diseases that produde systemic inflammation

  • Chronic infection
  • Chronic autoimmune disorders
  • Neoplasms; bronchogenic carcinoma, HL
92
Q

Erythropoietin level in anemia of chronic disease?
Serum ferritin?
TIBC?

A

low
high
low

93
Q

size and color of RBCs in anemia of chronic disease

A

Normocytic normochromic

94
Q

What type of anemia exhibits pancytopenia

A

aplastic anemia

95
Q

What is necessary for diagnosis of aplastic anemia

A

bone marrow biopsy: HYPOcellular, contains mainly fat

96
Q

Prognosis of Aplastic anemia

A

Bone marrow transplantation is treatment of choice and 5 year survival more than 75%

97
Q

A primary marrow disorder in which only red cell precursors are affected

A

Pure red cell aplasia

98
Q

Etiology of Pure red cell aplasia

A
  • thymoma: resection leads to improvement in half
  • Large, granular lymphocytic leukemia
  • certain drugs
  • autoimmune disorders
  • Parvovirus B19
99
Q

Abnormally high red cell count, increased Hgb

A

Polycythemia

100
Q

What is relative polycythemia

A

reduced plasma volume (hemoconcentration)

101
Q

erythropoietin in Primary absolute polycythemia?

Secondary

A

Low

high

102
Q

What is the most common cause of Primary absolute polycythemia?

A

polycythemia vera

Heme and Lymph (27 decks)

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