Heme 2 Flashcards
(126 cards)
1
Q
what level constitutes neutropenia?
A
decreased neutrophil <1500
2
Q
With a neutrophil count <100 what increases sharply
A
infectious diseases
3
Q
when the neutrophil count is <500 there is impaired what?
A
impaired control fo endogenous flora
4
Q
when neutrophil count is <200 what is absent?
A
inflammatory process is absent
5
Q
pathophys behind neutropenia
A
Decreased production (Marrow) Increased sequestration (Spleen) Peripheral destruction/pooling
6
Q
what drugs caues neutropenia via decreased production
A
sulfonamides (even bactrim) methotrexate PCN cephalosporins cimetidine phenytoin retrovirals
7
Q
benign condition where someone have drops in WBC periodically
A
cyclic neutropenia
8
Q
what ethnicity tend to has a lower WBC count?
A
african americans
9
Q
what nutrient deficiencies can cause neutropenia
A
folate or Vit B12 deficiency
10
Q
what infections can lead to neutropenia
A
TB measles mono viral hepatitis AIDS sepesis
11
Q
what causes peripheral destruction of neutrophils
A
anti-neutrophil antibodies
autoimmune disorders (RA, HIV, SLE)
Felty’s syndrome
Wegner’s granulomatosis
12
Q
what is felty’s syndrome
A
immune neutropenia + sero (+) nodular RA+splenomegaly)
13
Q
chronic inflammation + granuloma formation in the nasal pharynx, lung and kidneys
A
Wegner’s granulomatosis
14
Q
what cause neutropenia via peripheral pooling
A
severe bacterial infections
cardiopulmonary bypass
hemodialysis
sequestration (in spleen or liver)
15
Q
most common infections that occur due to neutropenia
A
pneumonia
cellulitis
septicemia
16
Q
clinical presentation of neutropenia
A
infection
stomatitis
fever
17
Q
why is a peripheral smear important
A
confirms what your instrument read
18
Q
when is a bone marrow biopsy needed w/ neutropenia
A
really low count and no known reason
19
Q
tx for felty’s syndrome w/ repetitive infections
A
splenectomy
20
Q
what can you do to tx immune mediated conditions causing neutropenia?
A
immune mediated therapy
21
Q
A rise in cell count above the normal limits of any leukocyte (myeloid or lymphoid lines).
A
leukocytosis
22
Q
what is neutrophilia
A
absolute count >10,000
23
Q
what are primary causes of neutrophilia
A
congenital disorders
myeloproliferative disorders
leukemia
24
Q
what are secondary causes of neutrophilia
A
infection (acute/ chronic) acute stress drugs steroids inflammation marrow hyperstimulation splenectomy smoking (mild)
25
how do you screen for chronic leukemia (chronic myelogenous leukemia)
BCR/ABL
26
what is BCR/ABL?
genetic mutation
27
what causes lymphocytosis
reactive increase to viral or fungal infection
allergic rxn
benign autonimmune process
lymphoplasmacytic disorders (luekemia CLL, ALL)
28
Decrease in platelet count below low normal (<150,000/μL).
thrombocytopenia
29
3 main mechanisms of thrombocytopenia
```
Decreased marrow production
Increased sequestration (in spleen)
Accelerated peripheral destruction
```
30
what caused decreased marrow production leading to thrombocytopenia
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
31
what is a very common cause of low platelets
alcohol abuse
32
what conditions can cause splenomegaly
Advanced liver disease
Myeloproliferative disorders
Malignancies of the spleen
33
how many platelets are held in the spleen at once
30%
34
most common cause of thrombocytopenia (immune mediated)
ITP
35
what is HIT?
heparin immune mediated thrombocytopenia
| usually the 2nd time they get heparin
36
what are non-immune mediated causes of thrombocytopenia
```
TTP/HUS
DIC
HELLP
anti-phospholipid syndrome
pre-eclampsia
```
37
what is HELLP
hemolytic anemia, elevated liver function tests, and low platelet count
38
how do people w/ thrombocytopenia present
```
mucosal or dermatologic bleeding
epistaxis
gingival bleeding
GI
purpura/ petechiae
```
39
labs for thrombocytopenia
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
40
is an autoimmune disorder which IgG antibodies are formed that bind to platelets which lead to destruction by the spleen
ITP
41
presentation with ITP
Overall, patients are well and afebrile.
Major complaint is mucosal or skin bleeding
More common in children than adults
42
what does ITP commonly follow in children
a viral illness
| commonly self limited in children
43
where does ITP tend to occur?
weight dependent areas
| look on ankles
44
does ITP blanch?
No
45
why are platelets slightly enlarged w/ normal morphology w/ ITP
newer platelets so they are larger
46
what is evan's syndrome
Blood smear show anemia, reticulocytosis and spherocytes
47
first line of tx w/ ITP
prednisone
48
what are other txs for ITP?
IVIG, Winrho (Rho-Gam) Rituxan
49
definitive tx for ITP
splenectomy
50
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
Nplate (Romipostim)
51
what is TTP?
Thrombotic Thrombocytopenic Purpura
| Non-Immune cause of thrombocytopenia accompanied by microangiopathic hemolytic anemia and increased serum LDH
52
what is the pentad w/ TTP
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
lab findings w/ TTP
anemia
reticulocytosis
smear shows microangiopathic picture
54
Tx for TPP
plasmapharesis
| prednisone and ASA as adjunct
55
what is the definitive test for TTP?
ADAMTS13 (protease) is low
| takes 2 weeks for result
56
More common cause of non-immune platelet destruction
DIC
57
what causes DIC
```
sepsis
severe tissue injury (excessive surgery)
obstetric complications
cancer
severe hepatic illness
```
58
labs for HUS
```
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
what should raise a flag for DIC?
Spontaneous bleeding or oozing following needle stick should raise a flag
60
tx for DIC
fibrinogen with cryopercipitate
fresh frozen plasma
platelet transfusion
61
if you give RBCs what else do you need to do?
give FFP and platelets too
62
WBCs may increase to 10-25,000
| RBCs usually normal
thrombocytosis
63
DDx for thrombocytosis
Polycythemia vera
reactive thrombocytosis
CML
64
complications w/ thrombocytosis
thrombosis and or embolus
stroke, MI, DVT, PE
hemorrhage may be severe
65
tx for thrombocytosis
ASA
| anticoagulation (plavix)
66
what do you use to treat essential thrombocytothemia (thrombocytosis)?
hydroxyurea
anagrelide
interferon
67
Excess production of all cell lines, esp. RBC
polycythemia vera (PCV)
68
Primarily excess platelet production
Essential Thrombocythemia (thrombocytosis)
69
Fibrosis of marrow with decreased cell production
myelofibrosis
70
1st presenting sign of polycythemia vera
splenomegaly
| Pruritis with warm water exposure
71
if left unchecked what can happen w/ polycythemia vera?
Neurological symptoms
Systolic BP increased ? viscosity
Arterial/Venous thrombosis can be a clinical indicator
Paradoxical presentation
72
test for polycythemia vera?
Jak 2 positive
73
tx for polycythemia vera
phelbotomy
low diet in Fe can be helpful
myelosuppressive therapy
74
should you put someone with polycythemia vera on iron even if levels are low?
NO!
75
what is a tx option for polycythemia treatment in older patients
hydroxyurea
76
what other drugs may be needed w/ polycythemia vera
allopurinol
| anithistamines
77
Uncommon Myelo Proliferative Disorder of unknown cause with marked proliferation of megakaryocytes in the bone marrow leading to high platelet count
Essential Thrombocytothemia
78
most common presentation of essential thrombocytothemia (thrombocytosis)
Thrombosis (random blood clots) commonly occur in mesenteric, hepatic, or portal vein
79
what will bone marrow look like for essential thrombocytothemia (thrombocytosis)
increased numbers of megakaryoctyes w/ normal morphology
80
Tx for thrombocytothemia (thrombocytosis)
hydroxyurea (want platelets <500,000)
| Anagrelide (poorly tolerated)
81
ADRs w/ anagrelide (tx for essential thrombocytothemia (thrombocytosis)
HA, mild anemia, peripheral edema
| CHF w/ high doses
82
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
Myelofibrosis
83
where does hematopoiesis take place with myelofibrosis
the liver, spleen and lymph nodes (as compensation)
84
how does someon with myelofibrosis present
Over 50
| fatigue (anemia), abdominal fullness, splenomegaly, hepatomegaly (50%)
85
Whey does portal HTN occur later in the course w/ myelofibrosis?
secondary to hepatic hematopoiesis
86
What will the blood smear look like w/ myelofiborsis (triad)
poikylocytosis
luekoerythroblastic blood
giant degranulated platelets
87
Tx for myelofibrosis
transufions, splenectomy
| alpha interferson
88
what helps to reduce spleen size w/ myelofibrosis
jakafi
89
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
Acute Lymphoblastic Leukemia (ALL)
90
who is mostly affected by ALL
young children (most common childhood malignancy)
91
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%)
Chronic lymphocytic leukemia (CLL)
92
what is the most common form of chronic luekemia?
CLL
93
Presentation of CLL
Complaints of weakness, fatigue, Dyspnea On Exertion, infections
PE: lymphadenopathy, hepatosplenomegaly
94
What chromosome abnormality may a patient with CLL Have?
trisomy 12
95
What is the staging system used for CLL?
Binet
96
CLL stage lymphocytosis w/10 yrs. No tx if asymptomatic
Binet Stage A
97
stage of CLL with anemia/thrombocytopenia despite number of lymph node groups; mean survival 2 yrs; Chlorambucil w/prednisone; splenic irradiation
Binet Stage C
98
CLL stage with 3 lymph node groups; Mean survival 5 years; Use alkylating agent and radiation to symptomatic nodes
Binet Stage B
99
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.
Acute Myelogenosu Leukemia (AML)
100
what is there a proliferation of with AML?
abnormal myeloid cells (precurosrs) that don't mature
101
How is AML classified?
FAB (7 subtypes)
102
Leukemia that presents w/ recurrent infection, bone pain, bruising, bleeding, weakness, bone tenderness.
AML
103
what is pathognomic for AML on labs
Auer rods
104
Peak 30-50 yrs, etiology unknown
Proliferation of immature granulocytes
has chronic and acute phases.
Chronic Myelogenous Leukemia (CML)
105
what phase of CML is where WBC >100,000/mm3 w/ <5% blasts
chronic phase
106
What phase of CML is where phase >5% blasts and more malignant phase w/WBC increase w/anemia and thrombocytopenia
acute blastic phase
107
what is present on labs w/ CML
Philadelphia chromosome
108
Tx for chronic phase of CML
tyrosine kinase inhibitors
109
```
Malignant disorder of lymphoreticular system
Characterized by
“Reed-Sternberg” cells
Bimodal age distribution
20-40 year olds
> 50 year olds
```
Hodgkin's Lymphoma
110
Complain of painless lump (in neck), fever, drenching night sweats, weight loss, (B symptoms) malaise, generalized pruritis
Hodgkin's lymphoma
111
what may cause pain in lymph nodes w/ hodgkin's lymphoma?
ETOH
112
What will be elevated on labs w/ hodgkins?
ESR and LDH
113
what will be decreased on labs w/ hodgkins?
lymphocytes and platelets
114
stage of hodkin's with 2 or more lymph node regions on same side of diaphragm
Stage II
115
Stage with Hogdkin's with diseeminated involvement of one or more extra-lymphatic organs w/ or w/o node involvement
Stage IV
116
Stage of Hodgkin's w/ single lymph node region involvement or single extra-lymphatic site.
Stage I
117
Stage of Hodgkin's w/ lymph nodes regions on both sides of diaphragm
Stage III
118
Tx for stage I-II of Hodgkin's
2 cycles ABVD chemo + XRT
119
Tx for stage III, IV of Hodgkin's
chombo chemo + radiation to sites of bulky dz
120
Malignancy of the lymphoreticular system
Classified as B, T or U (undifferentiated)
median age is 50
increased incidence w/ HIV
Non-hodgkin's lymphoma
121
Non-hodgkin's presentation
Persistent, painless lymphadenopathy in 2/3 of patients
| pruritis (rare)
122
Tx for Hodgkin's
Chemo + radiation, depends on stage
123
```
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
```
Multiple myeloma
124
presentation of multiple myeloma
```
Bone pain (back and fibs), pathological fractures
weakness, fatigue, N/V, bleeding, bruising
```
125
how is dx of multiple myeloma made?
serum or urine protein electrophoresis then confirmed w/ bone marrow
126
Tx for multiple myeloma
chemo, radiation, prednisone
