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Heme Week 2 > Elevated Blood Counts > Flashcards

Elevated Blood Counts Flashcards

1
Q

What myeloproliferative diseases can cause neutrophilia?

A
  • CML
  • PV
  • ET
  • Myelofibrosis
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2
Q

What are some non-myeloproliferaitve causes of neutrophilia?

A
  • cigarette use
  • obesity
  • drugs (corticosteroids/lithium)
  • infection/inflammation
  • malignancy

COD-MIM

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3
Q

What are some causes of increased platelets?

A
  • inflammation
  • trauma
  • malignancy
  • iron deficiency
  • splenectomy
  • myeloproliferative neoplasm (ET/MF/PV/CML-all of them)

ITS-MIM

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4
Q

What are myeloproliferative neoplasms?

A

hematopoietic neoplasms in which a clone of cells is mutated early in the differentiation of blood cells, such that these cells can differentiate fully into red cells, platelets and neutrophils, BUT no longer are well controlled in number, thus producing too many of one or more cell lines with fairly normal appearing and fairly normal functioning cells.

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5
Q

Central to MPNs is what?

A

understanding of the EPO receptor that is a JAK/STAT system which uses JAK-2 kinase

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6
Q

Mutated JAK2 kinase leads to what?

A

continuously active EPO pathway with or without EPO presence

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7
Q

A JAK2 mutation is most common in which MPN?

A

PV (90+%)

ET and Primary MF may have JAK2 mutation in up to 60% of cases and if they don’t, they have a mutation in MPL (TPO receptor) or CALR (calreticulin)

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8
Q

What happens in PV?

A

too many red cells which leads to increased plasma volume, dilation of the veins, and other symptoms

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9
Q

What are the most common physical findings of PV?

A

From most to least:

  • splenomegaly
  • skin plethora
  • conjunctival plethora
  • engorged retinal vessels
  • hepatomegaly
  • hypertension (systolic high more often)

H-CHESS

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10
Q

What are the most common symptoms of PV?

A
  • headache
  • weakness
  • pruritus
  • dizziness
  • diaphoresis (excess sweating)
  • visual disturbances
  • weight loss
  • parethesias

PV hurts 2 people, dogs, and daughters

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11
Q

What are common complications/causes of death in PV patients?

A
  • thrombosis/embolism (very common)
  • progression to AML
  • progression to myelofibrosis (become pancytopenic)
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12
Q

What is the median survival time from start of treatment (phlebotomy) in PV patients?

A

14 yrs

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13
Q

What is commonly seen in a PV blood smear?

A
  • too many platelets

- hypochromic microcytic cells due to iron deficiency (GI blood loss is common)

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14
Q

What does the bone marrow look like in PV patients?

A
  • very hyper cellular

- clusters of megakaryocyes

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15
Q

What are some risk factors for thrombosis in PV patients?

A
  • age 65+
  • previous thrombosis
  • WBC 15K+
  • CV risks
  • Elevated HCT

W-CAPE

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16
Q

How is PV diagnosed?

A

1) Hemoglobin greater than 18.5+ in men, 16.5 in women
2) Presence of JAK2617V mutation or JAK2 exon 12 mutation
3) EPO below normal range

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17
Q

How is PV treated?

A

1) Phlebotomy
Target HCT 45% (prevents thrombosis well)

2) Aspirin
100 mg/d significantly lowers combined risk of CV death, non-fatal myocardial infarction, non-fatal stroke, major thromboembolism (hazard ratio 0.4). Relative risk increase in major bleeding with ASA 1.6.

3) Hydroxyurea
Very effective in reducing thrombosis (1.6% vascular events/yr vs 10.7%)
Target HCT of 45 with WBC >3000, supplement with phlebotomies if needed
No clear increase in leukemic transformation

4) Interferon
3 million units/d until response and then lower
Peg-interferon 0.5 mcg/kg weekly increased to 1.0 if no response in 12 weeks
Blood 112:8 3065—35/40 CR at I year
Can control platelets and HCT in Majority of patients and reduce spleen size and alleviate pruritus
May work when Hydrea fails
Busulfan reasonable in elderly patients

5) JAK2 inhibitors

P-HAJI

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18
Q

Symptoms of ET:

A

-erythromelalgia (severe burning pain of the forefoot)

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19
Q

What is commonly seen in the bone marrow (and sometimes the blood smear) in ET?

A

large numbers of megakaryocytes

20
Q

How is ET diagnosed?

A

Platelets elevated (450 K/ul) without red cell elevation or iron deficiency and positive JAK2 mutation. IF JAK2 not positive, look in bone marrow for MPL or calreticulin mutation

21
Q

If platelets get above 1.5 million, what can happen?

A

they may acquire vmF diseases with loss of large vFM multimers resulting in bleeding (purpura)

22
Q

For major bleeding events in ET (can happen), what is the treatment?

A
  • Correct thrombocytosis

- Withdraw aspirin

23
Q

What is the treatment for ET?

A

need to reduce platelets:

  • Hydroxyurea (affects all cell lines-leads to pancytopenia)
  • Anagredile
  • Interferon (pregnancy risk)

HAI

24
Q

What does Anagredile do?

A

inhibits megakaryocytes= less platelets

25
Q

Side effects of Anagredile?

A
  • increased arterial thrombosis risk
  • major bleeding
  • myelofibrotic transformation

BAM

only used when Hydroxyurea not working

26
Q

What are the criteria for “high risk” for ET and should be treated with platelet reduction?

A
  • 60+ y/o
  • platelets above 1500K/ul
  • previous thrombosis, erythromelagia
  • diabetes/HTN

APED-1500

27
Q

What happens in primary MF?

A

fibrosis in BM, hematopoiesis is crowded out and moves to spleen and liver thus producing splenomegaly

28
Q

What other MPN causes splenomegaly?

A

CML

29
Q

What are some common findings in primary MF?

A
  • hepatosplenomegaly
  • fatigue and anemia
  • leukocytosis
  • thrombocytosis or -penia

H-FLAT

30
Q

MF can also arise from other things and is called ‘secondary’ MF. What neoplastic conditions can lead to secondary MF?

A
  • PV, CML
  • acute megakaryoblastic leukemia
  • myelodysplasia with fibrosis
  • many more

PAM-CML

31
Q

What non-neoplastic conditions can lead to secondary MF?

A
  • granulomatous disease
  • paget disease
  • hypo/hyperparathyroidism
  • many more

great post high

32
Q

What is the key findings in a peripheral smear of a primary MF patient?

A
  • tear drop cell (dacrocyte) non-specific

- giant platelets

33
Q

How is primary MF diagnosed?

A

Patient comes in with constitutional symptoms, spleen is enlarged, check bone marrow and check for JAK2, calreticulin, and Mpl mutations

34
Q

How is primary MF treated?

A

Allo SCT or JAk2 inhibitors

35
Q

What MPN is Philadelphia chromosome positive?

A

CML t(9,22) ABL-BCR

36
Q

What is CML?

A

condition where you have way too many white cells and you develop early white cells in the peripheral blood. Blood smear shows early neutrophils, particularly myelocytes

37
Q

How would a CML patient present?

A

with elevated neutrophil count, usually not ill. Often a palpable spleen

38
Q

How is CML diagnosed?

A

Diagnosis made by FISH or pcr for bcr-abl fusion transcripts,

Or by bone marrow with symptoms present and cytogenetics for 9:22 translocation

39
Q

What is the prognosis for CML?

A

Used to Be: Prognosis was 3-4 years survival with 25% converting to AML each year; allogeneic transplant was only cure and treatment of choice for those able.

Now we have tyrosine kinase inhibitors (Imatinib)

40
Q

How is CML treated? Goals of treatment?

A

Initial therapy is with Imatinib

Goal is complete cytogenetic response with no evidence of Philadelphia chromosome, or major molecular response with a 3 log reduction of bcr-abl transcripts by pcr technique

41
Q

What is hypereosinophilia caused by?

A
  • hypersensitivity
  • parasites
  • hypereosinophilic syndrome
42
Q

How is hypereosinophilic syndrome defined?

A

greater than 1500 level of eosinophils for over 6 months and start to see heart and lung damage

43
Q

What are some possible causes of Hypereosinophilic syndrome?

A
  • idiopathic
  • chronic eosinophilic leukemia
  • hematopoietic neoplasms accompanied by eosinophilia (T cell clonal disorders, AML, MPN-eos, MDN-eos)
44
Q

What are some complications of hypereosionphilic patients?

A 
myocarditis
lung infiltration
enteritis
encephalopathy, neuropathy
thromboses
eczema, angioedema

ELEMENT A

45
Q

What happens if you find a PDGFRa/b mutation by FISH or RT-PCR in an eosinophilic patient?

A

try treating with Imatinib

if organ damage is present: create with glucocorticoids, hydroxyurea, intereferon

Heme Week 2 (18 decks)

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