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Hematology 2 > test 1 info ( lectures 1-5) > Flashcards

test 1 info ( lectures 1-5) Flashcards

1
Q

neutrophil myeloblast

A

0-2% in BM
15-20µm
7:1- 5:1

no granules
no aggregation of material
1-3 nucleoli

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2
Q

Promyelocyte

A

1-4% in BM
12-24 µm
5:1- 3:1

primary granules 
slight aggregation of nucleus 
larger 
1-2 nucleoli 
more basophilic
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3
Q

myelocyte

A

5-20% in BM
1–18µm
2:1- 1:1

secondary granules
may have some primary granules
may or may not have nucleoli
last stage to divide

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4
Q

Metamyelocyte

A

5-15% in BM
10-18µm
1:1

indented kidney bean nucleus
basophilic chromatin

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5
Q

Band

A

10-35% in BM
10-16µm
1 : 1 - 1: 2

elongated nucleus ( horseshoe, S or U shaped) uniform thickness
basophilic chromatin
no filaments

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6
Q

Segmented

A

5-15% in BM
10-16µm
1:3

2-5 distinct nuclear lobes connected by strands of chromatin/ filaments

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7
Q

tissue neutrophils

A

located in Bone marrow
not phagocytic
immobile end stage cells
increased in CML, myelocytic/monocytic leukemia & myelofibrosis

features:
- Ample cytoplasm that is light blue with fine lattice structure
- blunt pseudopods
- long cytoplasmic extensions that wrap around other cells
- nucleoli are usually conspicuous

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8
Q

Neutrophils (bands)

A

2-6% of WBCs
uniform nucleus
pink cytoplasm
fine violet/pink granules

released in response to infection

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9
Q

neutrophils ( segmented)

A

50-70% of WBCs
2-5 nuclear lobes
pink cytoplasm
fine violet/pink granules

found in BM, circulating pool, marginal pool & the tissues
life span once released from BM is 10-14 days
neutrophilia: >6.0 x10^9/L
neutropenia: <1.5 x10^9/L

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10
Q

Monocytes

A

2-9% of WBCs

  • largest WBC
  • macrophage in tissues
  • increased in inflammation: syphilis, tuberculosis, endocarditis, rheumatoid arthritis & celiac disease

features:
- gray-blue cytoplasm
- fine reddish-purple evenly distributed granules
- “ ground glass appearance”
- lacy delicate chromatin
- variation in nuclear shapes
- vacuoles may be present
- blunt pseudopods
- monocytosis count: >1.0 x10^9/L

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11
Q

Lymphocytes

A

20-44% of WBCs
increased in viral infections, batcerial infections, drug reactions, allergic reactoins, alloimmune reactions, & hyperthyroidism

features:

  • smal round nucleus
  • clumped chromatin
  • small amount of sky- blue cytoplasm
  • can have azurophilic granules

lymphocytosis:
absolute lymph count in adults: >4.0 x 20 ^ 9/L
absolute lymoh count in infants/children: >10.0 x 10 ^9/L

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12
Q

Lymphoblast ( L1, L2, L3)

A

L1

  • small
  • chromatin homogenous
  • regular nuclear shape
  • no visible nuclei
  • scanty cytoplasm

L2

  • large
  • nucleus is irregular with clefting & indentation
  • 1 or more large nucleoli
  • lots of cytoplasm

L3

  • large
  • finely stippled
  • regular nuclear shape
  • 1 or more prominent nucleoli
  • lots of strongly basophilic cytoplasm
  • prominent cytoplasmic vacuolization
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13
Q

Reactive Lymph

A
  • lymphocytes reacting towards an antigen
  • increased in viral infections, bacterial infections, drug reactions & miscellaneous causes

features:
- elongated
- cytoplasm scallops around the RBCs
- peripheral basophilia

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14
Q

Necrobiotic WBC

A
  • part of the normal physiological death of the cell
  • can be caused by tumors or basophilia
  • also seen in old patient samples
  • ” chocolate chip cookie”
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15
Q

Megakaryocytes

A

largest of the hematopoietic cells in the bone marrow
30-100µm

features:
- multilobulated nucleus
- coarse linear chromatin
- bluish cytoplasm
- small dense reddish - blue granules that fragment to form plts

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16
Q

Eosinophils

A

0-4% of WBCs

  • in the BM for 4 days, blood for 3-4 hrs & tissue for 8-12 days
  • motile
  • phagocytic
  • contains enzymes that neutralize substances produced in hypersensitivity reactions ( secret proteins)
  • increased in allergic reactions & parasitic infections

features:
- size of a neutrophil
- banded or bi-lobed
- pink cytoplasm
- orange/red granules

eosinophilia: >0.6 x10^9/L

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17
Q

Basophils

A

0-2% of WBCs

  • contains heparin & histamine which is released during allergic reactions
  • regulates inflammation
  • can bind IgE antibody with antigen
  • increases in CML, allergies, hypersensitivity reactions & inflammatory reactions

features:
- coarse blue-black granules that obscure the nucleus
- usually bi-lobed

basophilia: >0.2 x 10^9/ L

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18
Q

Plasma cells

A

-B lymphocytes that secretes antibodies

features:
- dark eccentric nucleus with wheel spoke pattern
- no nucleoli
- abundant deep blue cytoplasm
- clear area perinuclear zone next to the nucleus
- can have grape like vacuoles & crystalline structures

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19
Q

Russel Bodies

A

immune globulins manufactured by plasmacytes
- causes grape-like vacuoles in plasm a cells cytoplasm
appear as round globules that can be red/pink, blue or colorless

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20
Q

toxic granulation

A
  • fine to coarse increased basophilic granules in the cytoplasm of neutrophils in severe infections
  • dark purple to purplish black granules
  • normal granules are few to none
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21
Q

toxic vacuolization

A

small to medium- sized vacuoles in neutrophils with severe infections & toxemias

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22
Q

Dohle bodies

A
  • pale blue inclusions at the periphery of cytoplasm of neutrophils
  • common with burns, infections, trauma, neoplasms & pregnancy
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23
Q

Auer rods

A
  • reddish rod-shaped bodies or needles
  • alignment of primary granules found in the cytoplasm of a myeloblast or monoblast
  • shows that type of leukemia is myeloid instead of lymphoid
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24
Q

Faggot cells

A
  • cells found in M3 ( acute promyelocytic leukaemia)

- there is multiple auer rods in the cytoplasm which gives the appearance of a stack or bundle of sticks/rods

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25
Q

Barr body

A
  • a “drumstick” appendage made up of oval mass of dense chromatin attached to nuclear lobe by a single filament in neutrophils ( most common), eosinophils, or basophil
  • normal in females
  • incidence: 0.6-8.8%
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26
Q

Smudge cells

A
  • broken up cells that are smeared
  • usually associated with CML
  • most commonly a lymph
  • can be done by making smears as they are delicate
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27
Q

Pelger-Huet Anomaly

A
  • qualitative disorder
  • generally benign
  • rare autosomal, dominant inherited abnormality of the nuclei & chromatin
  • chromatin is coarse & condensed
  • nucleus of neutrophil is dumbell/barbell shaped ( bilobed or not at all )
  • 70-90% of neutrophils are affected
  • hyposegmentation
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28
Q

Hypersegmentation

A
  • larger than normal neutrophils with more than 5 lobes
  • can be due to megaloblastic anemias (B12 or folate deficiency) or hereditary hypersegmentation ( benign autosomal dominant disorder)
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29
Q

Chediak- Higashi syndrome

A
  • rare autosomal recessive condition with azurophilic granulation of all leukocytes
  • abnormal granulation is characteristic of defective chemotaxis
  • moderate neutropenia & thrombopenia
  • patient has partial or complete albinism

features:
- large green primary granules
- large reddish- purple secondary granules

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30
Q

Alders Anomaly

A
  • rare genetic disorder with dark staining coarse granules in granulocytes, monocytes & sometimes lymphocytes
  • inherited autosomal- recessive disorder pf mucopolysaccharidosis
  • associated with mucopolysachharidosis, Hunter’s & Hurler’s syndrome
  • granules are produced by precipitated mucopolysaccharides
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31
Q

May- Hegglin Anomaly

A
  • Congenital autosomal dominant disorder associated with decreased platelet production ( thrombocytopenia), giant platelets & variable neutropenia
  • granulocytes & monocytes have blue-staining cytoplasmic inclusions that resemble dohle bodies, except they are larger
  • granules may also be found in eosinophils & basophils
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32
Q

Infectious Mononucleosis Pathophysiology

A
  • caused by Epstein- barr Virus
  • EBV is herpesvirus also known as human herpesvirus4
  • EBV is the most common cause of mono
  • there is an overactive immune system in patients with infectious mononucleosis
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33
Q

infectious mononucleosis transmission

A
  • spread orally “ kissing disease”
  • when B cells & epithelial cells of the oropharynx are infected with EBV it causes the virus to shed in the saliva
  • infection of these cells makes it east for the virus to spread through bood to other B cells & lymphatic tissue
  • EBV binds to the CD21 receptors on B lymphocytes & some epithelial cells in the oropharynx
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34
Q

infectious mononucleosis ( affected)

A

high incidence of active disease in late teens - early 20s

35
Q

infectious mononucleosis symptoms

A
  • individuals may be asymptomatic for several weeks
  • incubation period is 3-6 weeks from infection with EBV to binding of the B cells & onset of symptoms
  • malaise, headache, fever, sore throat, swollen lymph nodes, fatigue, splenomegaly ( in 50% of cases), hepatomegaly & decreased appetite due to spleen compressing the stomach
  • can have tonsilitis ( can be enlarged with whiteish- greyish exudate on the tonsils; airway obstruction is possible)
  • Leukoplakia; white patches on the sides of tongue
  • early satiety: full before meal is done
  • self limiting & usually resolves in 2-4 weeks
  • may get autoimmune hemolytic anemia due to antobodies against “i” RBC antigen
36
Q

Infectious mononucleosis diagnosis

A
  1. serology testing
    - used to detect the presence of heterophiles Abs
    - they are antibodies produced by B cells against the EBV
    - “monospot”
    - note: patient may not have the antibody the first time tested but if still symptomatic & tested again can show antibody ( needs time to develop)
  2. throat swab
    - for differential diagnosis to see if the patient has strep throat
  3. CBC with differential
    - lymphocytosis, possibly 10-20 x10 ^ 9/L
    - reactive lymphocytes
    - 1st week may see neutrophilia. later 50-80% mononuclear cells
  4. virology
    - further investigation could include a special test for the specific EBV antibody panel
    - this is usually not needed
37
Q

infectious mononucleosis may be confused with

A
  • clinical presentation can be confused with strept throat
  • some patient with mono develop a rash when exposed to any antibiotic in the penicillin family
  • these patients will not respond to antibodies if given for treatment
38
Q

infectious mononucleosis treatment

A
  • rest
  • ibuprofen
  • fluids/nutrition
  • if airway is obstructed may need steroids to reduce the swelling
  • may need tonsillectomy
  • no contact sports for 3-4 weeks as there is a concern the spleen could rupture
39
Q

Chronic Myeloproliferative disorders

A
  • a group of malignant disorders characterized by excessive proliferation of hematopoietic cells in the bone marrow
  • Chronic Myeloproliferative Disorders: A group of heterogenous diseases that came from the expansion of the hematopoietic pluripotent stem cell resulting in overproduction of one or more of the formed elements of the blood
  • the hallmark physical finding of CMPDs is splenomegaly which occurs in 40-99% of patients depending on disorder
  • CML( chronic myelogenous leukemia) ; excessive production of granulocytes
  • PV(polycythemia vera) ; overproduction of RBCs
  • ET(essential thrombocythemia) ; overproduction of platelets
  • IMF( idopathic myelofibrosis) ; prominence of marrow fibrosis & extramedularry hematopoiesis in the liver & spleen
40
Q

Erythrocytosis

A

increase in the circulating RBCs producing an HCT more than 52% in males & 47% in females
- an elevated HCT is present in 2 major conditions : relative erythrocytosis & absolute erythrocytosis

41
Q

relative erythrocytosis

A

a decrease in the total blood volume with normal RBC levels but decreased plasma volume

  • RBCs are not increased but looks that way as HCT & HB are increased, this is due to the loss of plasma which concentrates the patient sample
  • usually, it can be managed by restoring the amount of plasma volume
  • can be the result of an acute or chronic state
42
Q

Acute plasma loss ( relative erythrocytosis)

A
  • marked loss of fluids from burns, diarrhea or diuretic therapy
  • decrease in fluid intake
  • redistribution in body fluids like acute trauma
43
Q

Chronic plasma loss ( relative erythrocytosis )

A
  • usually seen in stress erythrocytosis
  • presents in middle age
  • associated with anxiety
  • WBCs & plts are not increased
  • may have physical appearance of a polcythemic
  • no hepatosplenomegaly
44
Q

relative erythrocytosis expected results

A
  • Normal RBCs
  • Normal EPO
  • Normal WBC count
  • Normal plt count
  • Normal LAP score
  • Normal bone marrow
  • Decreased plasma volume
45
Q

Absolute erythrocytosis

A
  • Absolute red cell mass is increased

- Normal or slightly increased plasma volume

46
Q

primary cause of Absolute erythrocytosis

A

autonomous erythropoiesis or polycythemia (rubra) vera

  • PV is the primary cause
  • its a disorder with increased RBC production & normal/low EPO
47
Q

Secondary causes of absolute erythropoiesis

A
  1. Hypoxemia/DEcreased oxygen saturation
    - increased in RBCs to compensate for the decrease in 02
  2. HIgh altitude
  3. Heavy smoking
  4. CArbon monoxide poisoning
  5. Pulmonary Disease ( COPD)
  6. Cardiovascular Disease
  7. Autonomous erythropoietin production
    - tumors can cause EPO to be produced
  8. Post kidney transplant
48
Q

absolute erythropoiesis treatment

A

therapeutic phlebotomy

49
Q

Polycythemia Vera

A
  • also known as Polycythemia Rubra Vera

- “ ruddy” facial appearance ( ski mask formation of the face)

50
Q

Polycythemia Vera Pathology

A
  • a malignancy with excessive BM production of RBCs & an increase in the total RBC volume
  • Mutation in the JAK-2-gene ( linked to ionization radiation & exposure to toxins such as benzene )
  • when this occurs, there is activation of RBC production without EPO
  • this leads to increased RBC volume with low/normal EPO levels
  • increased HCT & HB are seen as well
  • Increase in lactate dehydrogenase as it spills out of destroyed RBCs
  • JAK-2 is involved with receptors for EPO, TPO, IL-3 & G-CSF
  • WBC & plts may increase to a lesser degree
  • increased blood viscosity
  • myelobfibrosis or acute myeloid leukemia may develop
  • anemia
  • extramedullary hematopoiesis
  • hypersplenism
51
Q

Polycythemia WBCs

A
  • increase in 80% of patients
  • immature forms not seen normally
  • Eosinophils & basophils can be increased
  • 1/3 have high LAP score ( 70%)
52
Q

polycythemia vera PLts

A
  • normal

- thrombosis in more than half the cases

53
Q

Polycythemia Vera Bone marrow

A
  • Hypercellular/Hyperplastic
  • Erythroid hyperplasia
  • increased megakaryocytes & myelopoiesis
  • granulocytic hyperplasia
  • fibrosis
  • increasued reticulin in post- polycythemic myelofibrosis & myeloid metaplasia
  • iron stores are often depleted
54
Q

polycythemia vera smear

A
  • may see nucleated RBCs & poikilocytes
  • N/N RBCs
  • but if they cant increase iron stores to compensate for the extra RBCs than Micro/Hypo
55
Q

Polycythemia Vera affected

A
  • individuals 55-70 years old
  • average age is 60
  • slightly more common in males
56
Q

Polycythemia Vera Symptoms

A
  • mental confusion, headache, dizziness, visual changes, tinnitus, paresthesia, weight loss, epigastric pain , gout, pruritus ( itchy skin), thrombosis, hemorrhage, plethora, hypertension, a mild to moderate degree of splenomegaly ( 75% of cases due to increased RBCs) and hepatomegaly ( 35% of cases due to increased plts)
57
Q

Polycythemia Vera Diagnosis

A
  • look at HB & HCT levels
  • Serum EPO
    • low/normal = primary
    • high= secondary
  • genetic testing to detect the presence of JAK-2 gene
  • Bone marrow findings consistent with pleomorphic megakaryocytes
58
Q

Polycythemia Vera treatment

A
  1. therapeutic phlebotomy
  2. Acetylsalicylic acid ( ASA) to reduce thrombosis
  3. Hydroxcyurea to reduce RBCs
  4. JAK-2 inhibitors
59
Q

Lipid ( Lysosomal ) storage disease

A
  • a group of inherited autosomal recessive diseases of metabolis
  • strategic metabolic enzymes are missing or deficient because of a single gene deletion
  • this causes the metabolic products (the enzymes in the lysosome normally would have broken down) to build up
  • Histocytes, monocytes, macrophages & the cells of the bone marrow, liver, spleen ^ lymph nodes are affected
  • there are usually large easily identifiable cells specific to each disease
    - these cells are usually found within the BM
  • there is no cure for lipid storage diseases
60
Q

Gaucher’s disease

A

enzyme deficient: beta-glucocerebrosidase

  • the most common lysosomal storage disease
  • caused by a deficiency of the enzyme
  • this causes buildup pf the glycolipid glucocerbroside in the lysosomes of the macrophages
  • single gene deletion occurs on chromosme 1
  • lipid filled Gaucher cells displace normal cells in BM, spleen, liver, lungs & CNS
61
Q

Gauchers disease affects

A

more common in Jewish people from eastern Europe

62
Q

Gaucher’s disease symptoms

A
  • Pancytopenia
  • Splenomegaly
  • Hepatosplenomegaly
  • bone lesions
  • osteonecrosis of the femoral head
  • Pulmonary issues
63
Q

Gaucher’s disease Identifying cell

A
  • called the “Gaucher cell”
  • the cytoplasm has a crumpled tissue paper look
  • wrinkled cytoplasm
  • It’s lipid laden
64
Q

Gaucher’s disease lab features

A
  • N/N or N/Hypo anemia
  • leukopenia
  • thrombopenia
  • increased serum acid phosphatase
  • PAS reaction is positive for carbohydrates
65
Q

Gaucher’s disease treatment

A
  1. enzyme replacement therapy
    - Glucocerebrosidase is currently done
    - helps with viseral & hematological manifestations
    - skeletal disease responds more slowly
    - pulmonary involvement is relatively resistant to the enzyme
  2. surgical care
    - partial & total splenectomy
  3. Bone marrow transplant
  4. Gene replacement
    - the permanent cure
66
Q

Nieman-Pick disease

A

enzyme deficient : Sphingomyelinase

  • caused by the deficiency of the enzyme
  • this causes a build up of sphingomyelin in the macrophages/ histocytic lysosomes
  • single gene deletion occurs on chromosome 11
  • the phagocytic cells of the BM, spleen, liver, lymph nodes & lungs are stuffed with droplets of the complex lipid giving them a fine vacuolation of foaminess to the cytoplasm
  • neurons of the CNS also become enlarged & vacuolated
67
Q

3 types of Nieman -pcik disease

A
  1. type A
    - manifests in infancy with massive visceromegaly & severe neurologic deterioration
    - lung diseases occur as well
  2. type B
    - no neurological disorders
  3. type C
    - dementia & depression
68
Q

Nieman pick disease affects

A
  • increased prevalance in Ashkenazi Jewish population

- affects girls more often than boys

69
Q

Nieman pick disease symptoms

A
  • growth slowed or delayed
  • hepatosplenomegaly
  • Lymphadenopathy
  • Pigmentation
  • Neurological involvement/ impairment (depending on type)
  • cherry-red spots in the macula of each eye ( ~1/3)
70
Q

Nieman pick disease identifying cell

A
  • called a “foam cell”
  • other names “ droplet” or “lipid - laden giant foam cell”
  • they are found within bone marrow, aspirate, tissues & organs
71
Q

Nieman pick disease lab features

A
  • PAS reaction id negative
72
Q

Tay Sachs disease

A

Enzyme deficient: Hexodsaminidase A ( hex-A)

  • caused by a deficiency of the enzyme
  • this causes a build up of GM2 gangliosides in the brain & other tissues
  • single gene deletion on chromosome 15
  • nerve cells on the brain & spinal cord are destroyed
73
Q

Tay Sachs disease affects

A
  • higher incidence in the Ashkenazi Jewish population
74
Q

Tay Sachs disease Symptoms

A
  • appear 3-6 months after birth
  • loss of motor control
  • atrophy of muscles
  • seizures
  • death usually by 4 years of age
  • cherry-red spot in the macula of eyes
  • Neurodegeneration
  • Physical & mental deterioration
  • Macrocephaly
  • Paralysis
    • usually NO hepatosplenomegaly seen
75
Q

Tay Sachs disease identifying cell

A

-“ onion skin” appearance to cell/lysosomes

76
Q

Mucopolysaccharidoses (MPS)

A
  • disorder that constitute a group of lysosomal storage diseases caused by a deficiency in one of the enzymes involved in the breakdown of mucopolysaccharides (MPSs)
  • rare genetic disorders with most being autosomal recessive
  • it causes the buildup of mucopolysaccharides or now called glucosaminoglycans ( GAG) in the lysosomes
  • they are chains of carbohydrates
  • products are found in the reticuloendothelial system ( spleen, BM. & liver), lymph nodes, blood vessels, brain, heart, connective tissues & urine
77
Q

Mucopolysaccharidosis symptoms

A
  • there are progressive disorders with multisystem involvement
  • there is a wide spectrum of severity from mild to severe
  • developmental delay
  • macrocephaly with thicken skulls
  • spinal cord compression
  • hearing loss
  • corneal clouding
  • abnormal gums/teeth & enamel
  • obstructive sleep apnea
  • heart disease, due to storage of GAG& secondary fibrosis making valves abnormal
  • joint stiffness
  • dysplastic hips
  • heptaosplenomegaly, most likely due to tissue accumulation of GAG
  • intestinal/umbilical hernias
  • skeletal disease, likley due to the distruption of groeth plates
  • airway disease, secondary to storage of GAG in soft tissue causing a “floppy trachea”
  • CNS issues, cause in unknown maybe due to secondary issues like inflammation
78
Q

Mucopolysaccaridosis diagnosis

A
  • initial screening includes a toludine blue spot test or turbidity test
  • enzyme assays are needed to diagnose
    -blood smears
    • see large granules in leukocytes, especially
      lymphocytes
    • Alder-Reilly bodies
    • Toludine blue stain may be used
79
Q

Mucopolysaccaridosis treatment

A
  1. Hematopoietic stem cell transplant (HSCT)- BM transplant
  2. Intravenous Enzyme Replacement Therapy (ERT)
    • requires weekly transufusions
    • expensive
    • IV administration of enzymes doesnt allow for crossing of the blood-brain barrier so treatment for CNS involvement is impaired
  3. Spinal ERT
  4. Gene Therapy
  5. Blood- brain barrier penetration of proteins
80
Q

Hunter’s syndrome- MPS ll

A

enzyme deficient: iduronate-2-sulfatase (I2S)

  • X-linked recessive disorder
  • female carriers usually are carriers with no evidence of the disease
  • GAGs buidup in the lysosomes
  • rare; 1 in 100, 000
81
Q

Hunter’s syndrome symptoms

A
  • onset between 1-3 years of age in the severe form
  • a multi-system disorder that has skeletal, physical, respiratory & airway involvement
  • in sever form the following CNS features are present:
    • Progressive cognitive impairement
    • Seizures
    • Hearing loss
      - Decreased night & peripheral vision loss
  • note: corneal clouding is typically absent in those with hunter’s syndrome
82
Q

Hurler’s Syndrome - MPS l

A

enzyme deficient: alpha-L- iduronidase

  • rare; 1 in 100, 000
  • autosomal recessive
  • can range from severe to mild symptoms with the mild or attenuated form being called Scheie MPS l
83
Q

Hurler’s syndrome symptoms

A
  • onset occurs before 6 months of age in the severe form
  • skeletal abnormalities
    -cognitive impairment
  • heart disease ( myocardial infraction )
  • respiratory problems
  • hepatosplenomegaly
  • coarse facial features “ gargoyle like”
    • flatten nasal bridge, elongated skull, prominent facial
      bones & widely spaced eye sockets
  • corneal clouding
  • developmental delays, usually fine at birth & takes 3-6 months to see symptoms
  • recurring UTIs
84
Q

Hurler’s syndrome treatment

A
  • bone marrow transplant
  • enzyme replacement therapy
  • supportive care

Hematology 2 (4 decks)

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