Hematology Flashcards
Immune Thrombocytopenic purpura mean age
6 years
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.
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
ITP causes
infections (HIV), Medications: sulfonamides, heparin, EtOH, quinidine/quinine, live vaccines
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.
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
Hemoglobins and chains
HbF : alpha2gamma2 (high in adults with sickle cell or beta thal)
HbA: alpha2beta2
HbA2: alpha2delta2
Methemaglobin
Where the iron is Fe3+ and cannot bind oxygen. Enzymes regularly can reverse this but not alsways.
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
Sites of hematopoeisis
yolk sac, liver, spleen then bone marrow.
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.
acute porphyria
nervous derangements: mental disorders like hallucinations, anxiety, depression, paranoia; abdom pain, vomiting, neuropathy.
Cutaneous Prophyria
Asthma, infantile eczema, helminth infections, recurrent urticaria, collagen vascular diseases, serum sickness, neoplasms, hyperthermia, iron deficiency anemia
Eosinophilia
Asthma, infantile eczema, helminth infections, recurrent urticaria, collagen vascular diseases, serum sickness, neoplasms, hyperthermia
First line txt for iron deficiency anemia
Oral ferrous sulphate
Spherocytes seen in…
ABO incompatibility ( but not Rh), heriditary ( small/dense), G6pD,
Howell Jowel bodies
Asplenia/hyposplenism
Target cells
Too little Hb compared to cytoplasm.
Liver disease, HbC, beta thalessemia
Adolescent. Generalized lymphadenopathy, fever, chills, night sweats, pruritis, wt loss, malaise.
Hodgkin lymphoma. Do a chest X-ray, afterwards do everything else.
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
Serum sickness disease characteristics
Type 3 hypersensitivity (immune complexes). Cellular infiltration and complement cause vasculitis. Duration reflects time antigen-Ig in serum.
Hyper segmented neutrophils and toxic granulation
Good early sign of folate deficiency. Toxic granulation in infection.
Part of eye where HLA B27 association with pathology is highest.
Uvea (uveitis)
Petechiael rash, shock, fever and rash DDx
meningitis, toxic shock syndrome, scarlet fever, kawasaki disease
central pontine myelinolysis
Characterized by acute paralysis, dysphagia, dysarthria and other neurological symptoms. Most commonly caused by rapid correction of hyponatremia.
Types of hyponatremia
Hypotonic Hyponatremia (3 types), hypertonic hyponatremia, and Pseudo/isotonic hyponatremia
Pseudohyponatremia
measurement error due to high TAGs or paraproteinemia
Hypertonic Hyponatremia
Due to high levels of glucose or mannitol, causing free water into vascular compartment, diluting the Na level.
Hypotonic Hyponatremia types
Hypovolemic, euvolemic, hypervolemic
Hypovolemic Hypotonic hyponatremia
extrarenal: diarrhea, burns, vomiting, sweat. Renal: diuretics, addison’s, salt wasting disease. Tx: isotonic saline over 24 hours. Caution: CPM.
Euvolemic Hypotnic Hyponatremia
Due to pain, nausea or SIADH.
Hypervolemic hypotonic hyponatremia
Edema in the presence of low intravascular volume. Causes: CHF, liver failure, nephrotic syndrome, hypocortisolemia, hypothyroidism.
The “T” to add to ALARMS
transient erythroblastopenia of childhood
Critical things to exclude in a child with anemia
leukemia, aplastic anemia, kernicterus, bad hemolytic disease, sickle cell, bleeding
Causes of Macrocytic anemia
Hypothryoid, liver disease, B12 deficiency (malnourished, pernicious anemia, vegans), Folate deficiency (malabsorb, malnourish, maldigest), Inherited??
Symptoms of anemia
fatigue, lethargy, irritable, pallor, SOBOE, feel cold, presyncope/palpitations
Ref flags with anemia
Weight loss/FTT, fatigue, splenomegaly, lymphadenopathy, abnormality in other cell lines
Amount of juice and milk for children considered dangerous
> 24 oz
SBP in children of concern
less than 70 + 2(age)
