Week 17 Blood III Flashcards

1
Q

Acute Leukemia Treatment

A
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2
Q

Treatment of CNS disease secondary to ALL

A
  • ## Intrathecal Chemotherapy
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3
Q

5 things to know about Acute Leukemia

A
  1. AML and ALL may occur at any age (All more common in kids and AML more common in adults)
  2. AML is more common than ALL
  3. AML and ALL are fatal without treatment (bone marrow failure leading to bleeding/infection)
  4. prognosis and best treatment depends on genetic profile
  5. 2 phases of treatment – remission induction and post remission (cure)
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4
Q

5 things to know about Myodysplastic Syndrome

A
  1. Disease of the older adult
  2. commonly presents as fatigue
  3. should be expected in older patients with macrocytic anemia
  4. patients with unexpected or severe cytopenia should be referred to a hematologist
  5. Active treatment is available and can improve outcomes (treatment to address the cytopenia (rhEpo), prevent or delay AML and prolong life HMA)
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5
Q

Neutropenia

A

Low Absolute Neutrophil Count (ANC)

  • can be very bad
  • 1< ANC <1.5 mild - minimal risk of infection
  • 0.5 <ANC<1 Moderate - moderate risk of infection
  • <0.5 severe - hgih risk of infection
  • <0.2 very severe - very high risk of infection
  • can be:
    • acute and transient or
    • chronic and persistent (3 months is the threshold)

Acute neutropenia

  • caused by some infections, viral bacterial or protazoal
  • associated with immune disorders, auto-immune diseases
  • can be caused by drugs (anticonvulsants, antipsychotics etc)
  • always consider if they started a new drug

Chronic neutropenia
- may be intrinsic to bone marrow (congenital - cyclic neutropenia OR Acquired - MDS)
- may be extrinsic to bone marrow (nutritional - malnutrition, B12, copper OR immune - RA, SLE, LGL )

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6
Q

Costovertebral Angle Tenderness

A
  • useful in physical exam
  • find costovertebral angle and push gently with 1 finger
  • no tenderness, tap gently
  • Often positive with peylonephritis (chronic inflammation)
  • sensitivity 21%
  • specificty 88%
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7
Q

Leukocytosis

A
  • High WBC count
  • neutrophilia
  • monocytosis
  • lymphocytosis
  • eosinophilia

Most common cause is acute infection
- fever as well suggests infection
- left shift and toxic granualtion suggests even more strongly acute infection

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8
Q

Neutrophilia

A

An increase in the number of peripheral neutrophils at least 2 standard deviations above the mean
- form of leukocytosis
- seen commonly in acute infection
- also seen in chronic infection
- goes up with cancer, drugs etc

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9
Q

Monocytosis

A

leucocytosis
- also with infections
- seen in cancer
- can be found with autoimmune diseases (ex lupus)
- can be found with advanced liver disease (cirrhosis)

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10
Q

Lymphocytosis

A
  • elevated with stress and terror
  • infections can cause
  • CLL
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11
Q

Eosinophilia

A

elevated Eosinophil count
- goes us with parasitic infection
- allergies
- could be malaria if coming back from Africa
- sometimes with cancers (hodgkins)

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12
Q

Left Shift

A

when immature neutrophils are found on a blood smear
- called left shift because we draw the progression of maturation with less mature on the left and more mature on the right
- steroids – can cause neutrophilia (increased mature not immature)
- acute infection – causes left shift including bands
- with severe infection (sepsis) – see mylocytes
- chronic myloid leukemia (CML) - extended left shift including blasts

DO NOT mistake for AML or ALL which would include only mature leukocytes and blasts not the whole array

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13
Q

WBC homeostasis - Response to infection

A

infection –> organism engulfed by phagocytic cells (neutrophils and macrophages) –> macrophages emit cytokines to recruit more inflammatory cells –> macrophages give off C-CSF and GM-CSF (promote growth of WBC) –> infection cleared

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14
Q

Eosinophilia

A
  • goes us with parasitic infection
  • allergies
  • could be malaria if coming back from Africa
  • sometimes with cancers (hodgkins)
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15
Q

WBC homeostasis - Response to infection

A

infection –> organism engulfed by phagocytic cells (neutrophils and macrophages) –> macrophages emit cytokines to recruit more inflammatory cells –> macrophages give off C-CSF and GM-CSF (promote growth of WBC) –> infection cleared

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16
Q

Myloproliferative Neoplasms MPNs

A
  • clonal disorders arising in HSC
  • indolent and chronic
  • excessive production of normal cells in blood and bone marrow

Four main MPNs.
- Chronic mylogenous leukemia (CML)
- polycythemia vera (PV)
- Essential thromobocythemia (ET)
- Primary myelofibrosis (PMF)
(last 3 are disorders of JAK2)

17
Q

Chronic Myloid Leukemia (CML)

A
  • rare clonal bone marrow disease
  • associated with philadelphia chromosome
    • BCR-ABL gene fusion (present in 100% of cases)
  • asymptomatic leukocytosis
  • extended left shift
  • presense of basophils
  • includes splenomegaly (may be massive)
  • blood film that looks like bone marrow has just entered the blood
  • disease of older adults (rarely seen in <30)
  • more in men than women

Natural History
- not treated –> accelerated development that culminates in blast crisis in 3 years

Treatment
- tyrosine kinase inhibitor
- imatinib
- Dasatinib
- nilotinib
- busitinib
- ponatinib
(resistance can occur)

18
Q

BCR-ABL

CML

A
  • translocation of t(9;22) philadelphia chromosome (causing BCR-ABL gene fusion) –> BCR-ABL protein is a tyrosine kinase with altered specificity –> phosphorylation causes CML

CML
- Presence of BCR-ABL is the gold standard diagnosis of CML

CML Therapy
- specific tyrosine kinase inhibitors are used to treat CML
- drugs bind the ATP binding pocket of BCR-ABL prevents acquisition of Phosphate for phsophorylation –> phsophorylation cannot occur and leukemia is not caused

-

19
Q

Polycythemia Vera

A
  • primary erythrocytosis
  • mutations in JAK2 or CALR
  • splenomegaly
  • hgih hemoglobin
  • Thrombosis risk (think, blood so thick it doesnt flow)
  • 5% of these patients go on to develop CML
  • treat with aspirin and phlebotomy (bleed them out)
20
Q

Erythrocytosis

A

primary - polycythemia vera
- activating mutation of JAK2 (its on all the time, constantly causing RBC development)

secondary erythrocytosis
- caused by problem in heart or lung
- reduced SaO2 –> blood gets to kidneys and stimulates release of Epo –> Epo goes to bone marrow, activated JAK2 and
- low –> increased erythropoiesis

21
Q

Esential Thrombocythemia

A
  • increased platelets
  • mutation in JAK2 CALR MPL
  • big spleen
  • hight platelet count
  • some thrombosis and bleeding risk
  • very low AML risk

Treat with aspirin

22
Q

primary myelofibrosis

A
  • also a platelet disorder
  • mutations in JAK 2 and CALR
  • big spleen full of hematopoetic cells
  • constitutionak symptoms
  • early - moderate increase in WBC
  • late - pancytopenia (bone marrow becomes fibrotic bc of fibrotic factors secreated by megakaryocytes)
23
Q

Febrile Neutropenia

A
  • Medical Emergency (the more comorbidities an individual has the more dangerous this is)
  • scarce neutrophils –> eaily clearable infection can develop into sepsis
  • treat with broad spectrum antibiotics and then narrow afterwards

Approach to patient with Febrile Neutropenia

  • is patient hypotensive?
    • YES - maintain BP –> Saline bolus –> ICU
    • NO -
    • physical exam, find site of infection
    • culture blood, urine, sputum, CXR
    • Initiate broad spectrim antibiotic therapy
24
Q

General Approach to All Emergencies

ABCDEFGH

A

A - airway/C-spine - patent? Protected?
B - breathing (is air going in and out?)
C - Circulation - pulse? BP?
D - disability - conscious, bleeding , pain?
E - Examine - quick survey: skin, imbs, lines, drains
F - Fluids - get an IV
G - Get
H - Help