Heme 2 Flashcards

1
Q

what level constitutes neutropenia?

A

decreased neutrophil <1500

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2
Q

With a neutrophil count <100 what increases sharply

A

infectious diseases

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3
Q

when the neutrophil count is <500 there is impaired what?

A

impaired control fo endogenous flora

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4
Q

when neutrophil count is <200 what is absent?

A

inflammatory process is absent

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5
Q

pathophys behind neutropenia

A
Decreased production (Marrow)
Increased sequestration (Spleen)
Peripheral destruction/pooling
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6
Q

what drugs caues neutropenia via decreased production

A
sulfonamides (even bactrim)
methotrexate
PCN
cephalosporins
cimetidine
phenytoin
retrovirals
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7
Q

benign condition where someone have drops in WBC periodically

A

cyclic neutropenia

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8
Q

what ethnicity tend to has a lower WBC count?

A

african americans

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9
Q

what nutrient deficiencies can cause neutropenia

A

folate or Vit B12 deficiency

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10
Q

what infections can lead to neutropenia

A
TB
measles
mono
viral hepatitis
AIDS sepesis
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11
Q

what causes peripheral destruction of neutrophils

A

anti-neutrophil antibodies
autoimmune disorders (RA, HIV, SLE)
Felty’s syndrome
Wegner’s granulomatosis

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12
Q

what is felty’s syndrome

A

immune neutropenia + sero (+) nodular RA+splenomegaly)

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13
Q

chronic inflammation + granuloma formation in the nasal pharynx, lung and kidneys

A

Wegner’s granulomatosis

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14
Q

what cause neutropenia via peripheral pooling

A

severe bacterial infections
cardiopulmonary bypass
hemodialysis
sequestration (in spleen or liver)

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15
Q

most common infections that occur due to neutropenia

A

pneumonia
cellulitis
septicemia

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16
Q

clinical presentation of neutropenia

A

infection
stomatitis
fever

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17
Q

why is a peripheral smear important

A

confirms what your instrument read

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18
Q

when is a bone marrow biopsy needed w/ neutropenia

A

really low count and no known reason

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19
Q

tx for felty’s syndrome w/ repetitive infections

A

splenectomy

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20
Q

what can you do to tx immune mediated conditions causing neutropenia?

A

immune mediated therapy

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21
Q

A rise in cell count above the normal limits of any leukocyte (myeloid or lymphoid lines).

A

leukocytosis

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22
Q

what is neutrophilia

A

absolute count >10,000

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23
Q

what are primary causes of neutrophilia

A

congenital disorders
myeloproliferative disorders
leukemia

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24
Q

what are secondary causes of neutrophilia

A
infection (acute/ chronic)
acute stress
drugs
steroids
inflammation
marrow hyperstimulation
splenectomy 
smoking (mild)
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25
Q

how do you screen for chronic leukemia (chronic myelogenous leukemia)

A

BCR/ABL

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26
Q

what is BCR/ABL?

A

genetic mutation

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27
Q

what causes lymphocytosis

A

reactive increase to viral or fungal infection
allergic rxn
benign autonimmune process
lymphoplasmacytic disorders (luekemia CLL, ALL)

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28
Q

Decrease in platelet count below low normal (<150,000/μL).

A

thrombocytopenia

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29
Q

3 main mechanisms of thrombocytopenia

A
Decreased marrow production
Increased sequestration (in spleen)
Accelerated peripheral destruction
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30
Q

what caused decreased marrow production leading to thrombocytopenia

A

suppression of megakaryocytopoiesis (marrow damage) from cytotoxic chemo, ETOH
destruction of normal stem cells
intrinsic defects of the stem cells
metabolic abnormalities affecting megakaryocyte maturation

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31
Q

what is a very common cause of low platelets

A

alcohol abuse

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32
Q

what conditions can cause splenomegaly

A

Advanced liver disease
Myeloproliferative disorders
Malignancies of the spleen

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33
Q

how many platelets are held in the spleen at once

A

30%

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34
Q

most common cause of thrombocytopenia (immune mediated)

A

ITP

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35
Q

what is HIT?

A

heparin immune mediated thrombocytopenia

usually the 2nd time they get heparin

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36
Q

what are non-immune mediated causes of thrombocytopenia

A
TTP/HUS
DIC
HELLP
anti-phospholipid syndrome
pre-eclampsia
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37
Q

what is HELLP

A

hemolytic anemia, elevated liver function tests, and low platelet count

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38
Q

how do people w/ thrombocytopenia present

A
mucosal or dermatologic bleeding
epistaxis
gingival bleeding
GI
purpura/ petechiae
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39
Q

labs for thrombocytopenia

A

CBC w/diff, LFTs, ANA, RF, HIV, TSH
PT/PTT (protime & partial thromboplastin time)
Blood smear: with particular attention to platelet morphology, size, clumping
B12 & Folate levels
Consider Bone Marrow in pts > 60

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40
Q

is an autoimmune disorder which IgG antibodies are formed that bind to platelets which lead to destruction by the spleen

A

ITP

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41
Q

presentation with ITP

A

Overall, patients are well and afebrile.
Major complaint is mucosal or skin bleeding
More common in children than adults

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42
Q

what does ITP commonly follow in children

A

a viral illness

commonly self limited in children

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43
Q

where does ITP tend to occur?

A

weight dependent areas

look on ankles

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44
Q

does ITP blanch?

A

No

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45
Q

why are platelets slightly enlarged w/ normal morphology w/ ITP

A

newer platelets so they are larger

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46
Q

what is evan’s syndrome

A

Blood smear show anemia, reticulocytosis and spherocytes

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47
Q

first line of tx w/ ITP

A

prednisone

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48
Q

what are other txs for ITP?

A

IVIG, Winrho (Rho-Gam) Rituxan

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49
Q

definitive tx for ITP

A

splenectomy

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50
Q

what is a treatment for ITP that is new. It is a growth factor and helps encourage bone marrow to make platelets. ONly works while you give it.
weekly injection

A

Nplate (Romipostim)

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51
Q

what is TTP?

A

Thrombotic Thrombocytopenic Purpura

Non-Immune cause of thrombocytopenia accompanied by microangiopathic hemolytic anemia and increased serum LDH

52
Q

what is the pentad w/ TTP

A
  1. Thrombocytopenia (bruising or purpura)
  2. Microangiopathic hemolytic anemia (anemia, jaundice and a blood film featuring evidence of mechanical fragmentation of red blood cells)
  3. Neurologic symptoms (fluctuating), such as hallucinations, bizarre behavior, altered mental status, stroke, or headaches
  4. Kidney failure
  5. Fever
53
Q

lab findings w/ TTP

A

anemia
reticulocytosis
smear shows microangiopathic picture

54
Q

Tx for TPP

A

plasmapharesis

prednisone and ASA as adjunct

55
Q

what is the definitive test for TTP?

A

ADAMTS13 (protease) is low

takes 2 weeks for result

56
Q

More common cause of non-immune platelet destruction

A

DIC

57
Q

what causes DIC

A
sepsis
severe tissue injury (excessive surgery)
obstetric complications
cancer
severe hepatic illness
58
Q

labs for HUS

A
Thrombocytopenia (usually less severe)
RBC fragmentation (a must have)
Elevated LDH/Negative Coomb’s
Coagulation tests normal
Some increase in fibrin degradation
Renal biopsy can be performed for Dx
Necrosis and thrombi
59
Q

what should raise a flag for DIC?

A

Spontaneous bleeding or oozing following needle stick should raise a flag

60
Q

tx for DIC

A

fibrinogen with cryopercipitate
fresh frozen plasma
platelet transfusion

61
Q

if you give RBCs what else do you need to do?

A

give FFP and platelets too

62
Q

WBCs may increase to 10-25,000

RBCs usually normal

A

thrombocytosis

63
Q

DDx for thrombocytosis

A

Polycythemia vera
reactive thrombocytosis
CML

64
Q

complications w/ thrombocytosis

A

thrombosis and or embolus
stroke, MI, DVT, PE
hemorrhage may be severe

65
Q

tx for thrombocytosis

A

ASA

anticoagulation (plavix)

66
Q

what do you use to treat essential thrombocytothemia (thrombocytosis)?

A

hydroxyurea
anagrelide
interferon

67
Q

Excess production of all cell lines, esp. RBC

A

polycythemia vera (PCV)

68
Q

Primarily excess platelet production

A

Essential Thrombocythemia (thrombocytosis)

69
Q

Fibrosis of marrow with decreased cell production

A

myelofibrosis

70
Q

1st presenting sign of polycythemia vera

A

splenomegaly

Pruritis with warm water exposure

71
Q

if left unchecked what can happen w/ polycythemia vera?

A

Neurological symptoms
Systolic BP increased ? viscosity
Arterial/Venous thrombosis can be a clinical indicator
Paradoxical presentation

72
Q

test for polycythemia vera?

A

Jak 2 positive

73
Q

tx for polycythemia vera

A

phelbotomy
low diet in Fe can be helpful
myelosuppressive therapy

74
Q

should you put someone with polycythemia vera on iron even if levels are low?

A

NO!

75
Q

what is a tx option for polycythemia treatment in older patients

A

hydroxyurea

76
Q

what other drugs may be needed w/ polycythemia vera

A

allopurinol

anithistamines

77
Q

Uncommon Myelo Proliferative Disorder of unknown cause with marked proliferation of megakaryocytes in the bone marrow leading to high platelet count

A

Essential Thrombocytothemia

78
Q

most common presentation of essential thrombocytothemia (thrombocytosis)

A

Thrombosis (random blood clots) commonly occur in mesenteric, hepatic, or portal vein

79
Q

what will bone marrow look like for essential thrombocytothemia (thrombocytosis)

A

increased numbers of megakaryoctyes w/ normal morphology

80
Q

Tx for thrombocytothemia (thrombocytosis)

A

hydroxyurea (want platelets <500,000)

Anagrelide (poorly tolerated)

81
Q

ADRs w/ anagrelide (tx for essential thrombocytothemia (thrombocytosis)

A

HA, mild anemia, peripheral edema

CHF w/ high doses

82
Q

characterized by fibrosis of the bone marrow, splenomegaly and leukoerythroblastic peripheral blood picture with poikylocytosis
Fibrosis of the marrow believed to be a response of platelet-derived growth factor and other cytokines

A

Myelofibrosis

83
Q

where does hematopoiesis take place with myelofibrosis

A

the liver, spleen and lymph nodes (as compensation)

84
Q

how does someon with myelofibrosis present

A

Over 50

fatigue (anemia), abdominal fullness, splenomegaly, hepatomegaly (50%)

85
Q

Whey does portal HTN occur later in the course w/ myelofibrosis?

A

secondary to hepatic hematopoiesis

86
Q

What will the blood smear look like w/ myelofiborsis (triad)

A

poikylocytosis
luekoerythroblastic blood
giant degranulated platelets

87
Q

Tx for myelofibrosis

A

transufions, splenectomy

alpha interferson

88
Q

what helps to reduce spleen size w/ myelofibrosis

A

jakafi

89
Q

Idiopathic malignant proliferation of lymphoid stem cells in bone marrow that invade lymph nodes, spleen, liver, and other organs
Classed as B or T cell

A

Acute Lymphoblastic Leukemia (ALL)

90
Q

who is mostly affected by ALL

A

young children (most common childhood malignancy)

91
Q

Usually in adults over 50 years
Male:Female (2:1)
proliferation of mature-appearing lymphocytes in peripheral blood that invade bone marrow, spleen, lymph nodes
Usually B-lymphocytes (>90%)

A

Chronic lymphocytic leukemia (CLL)

92
Q

what is the most common form of chronic luekemia?

A

CLL

93
Q

Presentation of CLL

A

Complaints of weakness, fatigue, Dyspnea On Exertion, infections
PE: lymphadenopathy, hepatosplenomegaly

94
Q

What chromosome abnormality may a patient with CLL Have?

A

trisomy 12

95
Q

What is the staging system used for CLL?

A

Binet

96
Q

CLL stage lymphocytosis w/10 yrs. No tx if asymptomatic

A

Binet Stage A

97
Q

stage of CLL with anemia/thrombocytopenia despite number of lymph node groups; mean survival 2 yrs; Chlorambucil w/prednisone; splenic irradiation

A

Binet Stage C

98
Q

CLL stage with 3 lymph node groups; Mean survival 5 years; Use alkylating agent and radiation to symptomatic nodes

A

Binet Stage B

99
Q

Primarily adults >50 yrs (can see in children)
Can be preceded by CML, PV, or other MPD and caused by chemo, chromosomal abn, radiation toxins, drugs or even idiopathic.

A

Acute Myelogenosu Leukemia (AML)

100
Q

what is there a proliferation of with AML?

A

abnormal myeloid cells (precurosrs) that don’t mature

101
Q

How is AML classified?

A

FAB (7 subtypes)

102
Q

Leukemia that presents w/ recurrent infection, bone pain, bruising, bleeding, weakness, bone tenderness.

A

AML

103
Q

what is pathognomic for AML on labs

A

Auer rods

104
Q

Peak 30-50 yrs, etiology unknown
Proliferation of immature granulocytes
has chronic and acute phases.

A

Chronic Myelogenous Leukemia (CML)

105
Q

what phase of CML is where WBC >100,000/mm3 w/ <5% blasts

A

chronic phase

106
Q

What phase of CML is where phase >5% blasts and more malignant phase w/WBC increase w/anemia and thrombocytopenia

A

acute blastic phase

107
Q

what is present on labs w/ CML

A

Philadelphia chromosome

108
Q

Tx for chronic phase of CML

A

tyrosine kinase inhibitors

109
Q
Malignant disorder of lymphoreticular system
Characterized by 
	“Reed-Sternberg” cells
Bimodal age distribution
20-40 year olds
> 50 year olds
A

Hodgkin’s Lymphoma

110
Q

Complain of painless lump (in neck), fever, drenching night sweats, weight loss, (B symptoms) malaise, generalized pruritis

A

Hodgkin’s lymphoma

111
Q

what may cause pain in lymph nodes w/ hodgkin’s lymphoma?

A

ETOH

112
Q

What will be elevated on labs w/ hodgkins?

A

ESR and LDH

113
Q

what will be decreased on labs w/ hodgkins?

A

lymphocytes and platelets

114
Q

stage of hodkin’s with 2 or more lymph node regions on same side of diaphragm

A

Stage II

115
Q

Stage with Hogdkin’s with diseeminated involvement of one or more extra-lymphatic organs w/ or w/o node involvement

A

Stage IV

116
Q

Stage of Hodgkin’s w/ single lymph node region involvement or single extra-lymphatic site.

A

Stage I

117
Q

Stage of Hodgkin’s w/ lymph nodes regions on both sides of diaphragm

A

Stage III

118
Q

Tx for stage I-II of Hodgkin’s

A

2 cycles ABVD chemo + XRT

119
Q

Tx for stage III, IV of Hodgkin’s

A

chombo chemo + radiation to sites of bulky dz

120
Q

Malignancy of the lymphoreticular system
Classified as B, T or U (undifferentiated)
median age is 50
increased incidence w/ HIV

A

Non-hodgkin’s lymphoma

121
Q

Non-hodgkin’s presentation

A

Persistent, painless lymphadenopathy in 2/3 of patients

pruritis (rare)

122
Q

Tx for Hodgkin’s

A

Chemo + radiation, depends on stage

123
Q
Malignant proliferation of plasma cells where of the 5 classes of immunoglobulin produced, one class is produced in excess
more often in men and AA
A

Multiple myeloma

124
Q

presentation of multiple myeloma

A
Bone pain (back and fibs), pathological fractures
weakness, fatigue, N/V, bleeding, bruising
125
Q

how is dx of multiple myeloma made?

A

serum or urine protein electrophoresis then confirmed w/ bone marrow

126
Q

Tx for multiple myeloma

A

chemo, radiation, prednisone