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Flashcards in Hematology Deck (39):
1

Immune Thrombocytopenic purpura mean age

6 years

2

ITP vs autoimmune thrombocytopenic purpura

ITP is in kids and is acute; ATP occurs in adults and is a chronic condition. OCcurs abruptly in children and 80% spontaneously resolve and gradual onset with rare remession in adults.

3

ITP

OFten follows infections (or live vaccine). Auto-Ig's against platelets seen, causing destruction in spleen. Kids: usually resolves in 2 months. Hemorrhage = major complication (ie head).

Signs: brusing/pupura, menorrhagia, epistaxis, gingival bleed. Nonpalpable spleen. Low platelets but all else normal.

Tx: IVIG, glucocorticoids, splenectomy
ddx: DIC, TTP, vWD, aplastic anemia, leukemia

4

ITP causes

infections (HIV), Medications: sulfonamides, heparin, EtOH, quinidine/quinine, live vaccines

5

TTP Presentation

acute or subacute onset. Fever, neurologic symptoms (mentat status, seizures, focal sx), fatigue, severe bleeding from thombocytopenia/purpura. Looks like HUS, but with neuro.

6

TTP characteristics

Rare (ADAMTS13 def, which breaks down vWF). Unknown etiology. Microangiopathic hemolytic anemia, thrombocytopenic purpura, fever, renal disease and neurologic disease.
Tx: plasma exchange with FFP

7

Hemoglobins and chains

HbF : alpha2gamma2 (high in adults with sickle cell or beta thal)
HbA: alpha2beta2
HbA2: alpha2delta2

8

Methemaglobin

Where the iron is Fe3+ and cannot bind oxygen. Enzymes regularly can reverse this but not alsways.

9

Abnormal Hb's

HbH: alpha thalassemia beta4
Hb Barts: gamma4 (alpha thal)
HbC, HbE: altered beta chain. Mild hemolytic anemia
HbAS: heterozygous sickle cell
HbSC: sickle HbC phenotype

10

Sites of hematopoeisis

yolk sac, liver, spleen then bone marrow.

11

Porphyria classification

By symptoms (acute vs cutaneous) and by physiologic site of accumulation (erythropoeitic vs hepatic).
Can color feces and urine purple during attack.

Consider/test in cases of Guillan Barre.

12

acute porphyria

nervous derangements: mental disorders like hallucinations, anxiety, depression, paranoia; abdom pain, vomiting, neuropathy.

13

Cutaneous Prophyria

Asthma, infantile eczema, helminth infections, recurrent urticaria, collagen vascular diseases, serum sickness, neoplasms, hyperthermia, iron deficiency anemia

13

Eosinophilia

Asthma, infantile eczema, helminth infections, recurrent urticaria, collagen vascular diseases, serum sickness, neoplasms, hyperthermia

14

First line txt for iron deficiency anemia

Oral ferrous sulphate

15

Spherocytes seen in...

ABO incompatibility ( but not Rh), heriditary ( small/dense), G6pD,

16

Howell Jowel bodies

Asplenia/hyposplenism

17

Target cells

Too little Hb compared to cytoplasm.
Liver disease, HbC, beta thalessemia

18

Adolescent. Generalized lymphadenopathy, fever, chills, night sweats, pruritis, wt loss, malaise.

Hodgkin lymphoma. Do a chest X-ray, afterwards do everything else.

19

Serum sickness presentation

Fever and malaise (100%) 1-3 weeks after first exposure (days if second exposure). Cutaneous eruptions, arthralgia, myalgia, GI symptoms, headache, blurred vision, dyspnea

20

Serum sickness disease characteristics

Type 3 hypersensitivity (immune complexes). Cellular infiltration and complement cause vasculitis. Duration reflects time antigen-Ig in serum.

21

Hyper segmented neutrophils and toxic granulation

Good early sign of folate deficiency. Toxic granulation in infection.

23

Part of eye where HLA B27 association with pathology is highest.

Uvea (uveitis)

24

Petechiael rash, shock, fever and rash DDx

meningitis, toxic shock syndrome, scarlet fever, kawasaki disease

25

central pontine myelinolysis

Characterized by acute paralysis, dysphagia, dysarthria and other neurological symptoms. Most commonly caused by rapid correction of hyponatremia.

26

Types of hyponatremia

Hypotonic Hyponatremia (3 types), hypertonic hyponatremia, and Pseudo/isotonic hyponatremia

27

Pseudohyponatremia

measurement error due to high TAGs or paraproteinemia

28

Hypertonic Hyponatremia

Due to high levels of glucose or mannitol, causing free water into vascular compartment, diluting the Na level.

29

Hypotonic Hyponatremia types

Hypovolemic, euvolemic, hypervolemic

30

Hypovolemic Hypotonic hyponatremia

extrarenal: diarrhea, burns, vomiting, sweat. Renal: diuretics, addison's, salt wasting disease. Tx: isotonic saline over 24 hours. Caution: CPM.

31

Euvolemic Hypotnic Hyponatremia

Due to pain, nausea or SIADH.

32

Hypervolemic hypotonic hyponatremia

Edema in the presence of low intravascular volume. Causes: CHF, liver failure, nephrotic syndrome, hypocortisolemia, hypothyroidism.

33

The "T" to add to ALARMS

transient erythroblastopenia of childhood

34

Critical things to exclude in a child with anemia

leukemia, aplastic anemia, kernicterus, bad hemolytic disease, sickle cell, bleeding

35

Causes of Macrocytic anemia

Hypothryoid, liver disease, B12 deficiency (malnourished, pernicious anemia, vegans), Folate deficiency (malabsorb, malnourish, maldigest), Inherited??

36

Symptoms of anemia

fatigue, lethargy, irritable, pallor, SOBOE, feel cold, presyncope/palpitations

37

Ref flags with anemia

Weight loss/FTT, fatigue, splenomegaly, lymphadenopathy, abnormality in other cell lines

38

Amount of juice and milk for children considered dangerous

>24 oz

39

SBP in children of concern

less than 70 + 2(age)