Hematology

Question 1

Immune Thrombocytopenic purpura mean age

Answer 1

6 years

Question 2

ITP vs autoimmune thrombocytopenic purpura

Answer 2

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.

Question 3

ITP

Answer 3

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

Question 4

ITP causes

Answer 4

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

Question 5

TTP Presentation

Answer 5

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

Question 6

TTP characteristics

Answer 6

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

Question 7

Hemoglobins and chains

Answer 7

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

Question 8

Methemaglobin

Answer 8

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

Question 9

Abnormal Hb’s

Answer 9

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

Question 10

Sites of hematopoeisis

Answer 10

yolk sac, liver, spleen then bone marrow.

Question 11

Porphyria classification

Answer 11

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.

Question 12

acute porphyria

Answer 12

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

Question 13

Cutaneous Prophyria

Answer 13

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

Question 13

Eosinophilia

Answer 13

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

Question 14

First line txt for iron deficiency anemia

Answer 14

Oral ferrous sulphate

Question 15

Spherocytes seen in…

Answer 15

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

Question 16

Howell Jowel bodies

Answer 16

Asplenia/hyposplenism

Question 17

Target cells

Answer 17

Too little Hb compared to cytoplasm.

Liver disease, HbC, beta thalessemia

Question 18

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

Answer 18

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

Question 19

Serum sickness presentation

Answer 19

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

Question 20

Serum sickness disease characteristics

Answer 20

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

Question 21

Hyper segmented neutrophils and toxic granulation

Answer 21

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

Question 23

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

Answer 23

Uvea (uveitis)

Question 24

Petechiael rash, shock, fever and rash DDx

Answer 24

meningitis, toxic shock syndrome, scarlet fever, kawasaki disease

Question 25

central pontine myelinolysis

Answer 25

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

Question 26

Types of hyponatremia

Answer 26

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

Question 27

Pseudohyponatremia

Answer 27

measurement error due to high TAGs or paraproteinemia

Question 28

Hypertonic Hyponatremia

Answer 28

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

Question 29

Hypotonic Hyponatremia types

Answer 29

Hypovolemic, euvolemic, hypervolemic

Question 30

Hypovolemic Hypotonic hyponatremia

Answer 30

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

Question 31

Euvolemic Hypotnic Hyponatremia

Answer 31

Due to pain, nausea or SIADH.

Question 32

Hypervolemic hypotonic hyponatremia

Answer 32

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

Question 33

The “T” to add to ALARMS

Answer 33

transient erythroblastopenia of childhood

Question 34

Critical things to exclude in a child with anemia

Answer 34

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

Question 35

Causes of Macrocytic anemia

Answer 35

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

Question 36

Symptoms of anemia

Answer 36

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

Question 37

Ref flags with anemia

Answer 37

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

Question 38

Amount of juice and milk for children considered dangerous

Answer 38

> 24 oz

Question 39

SBP in children of concern

Answer 39

less than 70 + 2(age)