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Hematology > Anemia > Flashcards

Anemia Flashcards

1
Q

Name non hematological manifestations of iron deficiency anemia (8)

A
  • Pica, pagophagia
  • Irritability, hyperactivity
  • Reduced cognition
  • Reduced work performance
  • Restless leg syndrome
  • Breath holding spells
  • Decreased cardiac output
  • Stroke
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2
Q

What is the iron challenge test?

A

Allows to evaluate if oral iron is absorption; Measurement of serum iron before and 2h after 1 mg/kg elemental iron

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3
Q

Name causes of reduced absorption of oral iron

A
  • Poor bioavailability: type of iron, cow milk, concomitant absorption of inhibitors (bran, tannins, …), high pH (antiacid, gastrectomy) - GI surface loss/dysfunction: celiac enteropathy, duodenectomy, short gut syndrome
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4
Q

What is the recommended monitoring after starting iron?

A

CBC/retics at 1-2 weeks for severe cases CBC at 2-3 months for all; should document resolution of anemia Continue treatment for at least 1 month following normalisation of CBC counts

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5
Q

DDx polycythemia (5)

A
  • Hypoxia: smoking, high altitude - Hb variants with high Hb affinity - Polycythemia vera: JAK 2 mutation - EPO receptor mutation - Elevation of EPO, can be 2nd to Chuvash mutation, VHL, HIF gene mutation
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6
Q

Polycythemia work up

A
  • SaO2 - CBC, smear - Serum EPO levels - Imaging: renal, posterior fossa - O2-Hb dissociation curve
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7
Q

Hemochromatosis: - Pathophysiology - Causes - Management

A
  • Causes: inherited (AR); most commonly HFE gene; juvenile form (HJV/HAMP) - Pathophysiology: decreased hepcidin levels, leads to iron accumulation - Management: monitoring iron studies, low iron diet, refrain from alcohol Therapeutic phlebotomy if Sx Beware of risk of thrombosis
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8
Q

Causes of folate deficiencies

A
  • Decreased intake: severe malnutrition, sick premature infant, unpasteurized goat milk - Intestinal malabsorption: celiac disease, IBD, anticonvulsants - Increased requiremements: pregnancy, chronic hemolytic anemia - Hereditary folate malabsorption, inborn errors of metabolism
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9
Q

Causes of B12 deficiency

A
  • Decreased intake: vegan (and their breastfed infant) - Decreased intestinal absorption (mediated by IF): Pernicious anemia, post-gastrectomy - Malabsorption (non-IF mediated): ileal resection, IBD, Imerslund-Grasbeck sd - Increased intestinal utilization: intestinal blind loop, fish tapeworm
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10
Q

Congenital pernicious anemia: - Inheritance - Diagnosis - Treatment

A
  • AR - Low serum B12 level, abnormal Schilling test, corrected by IF - IM B12
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11
Q

Imerslund-Grasbeck syndrome - Inheritance - Diagnosis - Treatment

A
  • AR, mutations leading to inability of IF/B12 to bind in terminal ileum - Low serum B12 level, abnormal Schilling test, not corrected by IF Proteinuria - IM B12
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12
Q

Transcobalamin II deficiency - Inheritance - Symptoms - Definitive diagnosis - Treatment

A
  • AR - Pancytopenia, diarrhea, FTT in early infancy - Low/absent serum TC-II level - Daily high dose IM B12
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13
Q

Lead poisoning - laboratory findings

A
  • Basophilic stippling in erythrocytes - Elevated free erythrocyte protoporphyrin - Only if concommitant IDA (pica): microcytic anemia
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14
Q

Which infants should receive supplemental iron?

A

Premature, LBW infants Neonate with anemia proven or suspected to be 2nd to external blood loss

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15
Q

Iron-resistant iron-deficient anemia: - Underlying cause

A

Homozygous deletions of matripase-2 genes (TMPRSS6) Increases hepcidin, leading o reduced dietary iron aborpstion and iron sequestration

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16
Q

What is basophilic stippling? Associated conditions (7)

A

Aggregated ribosomes with incompletely degrade RNA 1. Thalassemia 2. Hemoglobinopathies 3. Lead poisoning 4. Pyrimidine 5’ nucleotidase deficiency 5. Sideroblastic anemia 6. MDS 7. Congenital dyserythropoietic anemia

17
Q

What are Howell-Jolly bodies? Associated conditions (6)

A

Retained nuclear material 1. Post-splenectomy 2. Functional asplenia 3. Severe hemolytic anemia 4. Megaloblastic anemia 5. Cong dyserythropoietic anemia 6.Occasionally newborns, prematures

18
Q

What are Pappenheimer bodies? Associated conditions (2)

A

Lysosomes containing iron-protein complexes 1. Asplenia (anatomical or functional) 2. Iron overload

19
Q

What are Heinz bodies? How are they assessed? Associated conditions (4)

A

Aggregates of denatured hemoglobin, attached to inner red cell membrane Supravital stain, e.g. brilliant cresyl blue 1. G6PD deficiency 2. Hereditary Heinz body hemolytic anemia 3. Oxidant drug or chemical poisoning 4. Rare hemoglobinopathies

20
Q

What are hemoglobin H inclusions? How are they assessed? Associated condition (1)

A

Precipitates of excess beta-tetramer chains Supravital stain, e.g. brilliant cresyl blue 1. Alpha-thal with deletion of 3/4 genes

21
Q

Pelger-Huet anomaly: - Characteristic morphology - Associated mutation

A
  • Hyposegmented bilobed neutrophils (heterozygotes) or unilobed neutrophils (homozygous) - Lamin B receptor
22
Q

Diagnosis?

Bone marrow findings?

A

Gaucher disease;

Gaucher cells = reticuloendothelial cells (monocytic lineage) with lysosomes stuffed with glucosphingolipids

23
Q

Diagnosis?

Name of this finding

A

Mucopolysaccharidoses

Finding = Alder-Reilly anomaly (prominent azurophilic granulation); lysosoal bluid-up of mucopolysaccharides

24
Q

Hereditary stomatocytosis:

  • Underlying anomaly
  • Diagnostic features (5)
A
  • Mutations in Rh associated glycoprotein, which leads to cation permeable red cells with resultat overhydration (very icnreased intracellular Na)
  • Diagnostic features: peripheral smear stomatocytes (5-50%); hemolysis; macrocytosis; low MCHC; positive osmotic fragility test
25
Q

Indications of parenteral iron (4)

A
  1. Non tolerance of oral iron
  2. Compromised GI absorption of iron
  3. Need for rapid replacement of iron stores
  4. EPO therapy necessary
26
Q

Name side effects of parenteral iron (at least 2)

A
  1. Anaphylactoid reaction
  2. Mild to moderate arthralgias the following day
  3. Iron dextran: flares of rheumatoid arthritis
  4. IM: risk of subcutaneous leakage and skin discoloration
27
Q

Name indications for splenectomy in hereditary spherocytosis (4)

A
  • Severe anemia (usually defined as Hgb < 80g/L)
  • Chronic fatigue
  • Evidence of extramedullary hematopoiesis, e.g. frontal bossing
  • Poor growth
28
Q

What is the best specific diagnostic test for HS?

A

Eosin-5-maleimide (EMA) binding test

Uses flow cytometry to evaluate fluorescent probe EMA binding to Band 3

(detects all types of HS; 100% SE when combined with acidified glycerol lysis test)

29
Q

What is the acidified glycerol lysis test?

A

Autohemoylsis test, to diagnose HS

Method: incubation of RBC in hypotonic solution with glycerol (slows the entrance of water into the cells); Spherocytes from patients with HS lyse more quickly than normal red blood cells due to their reduced surface area to volume ratio.

30
Q

Which protein defects leads to HS?

A
  • Spectrin
  • Ankyrin
  • Band 3
  • Protein 4.2

(proteins forming the vertical cytoskeleton of RBCs)

31
Q

Describe treatment modalities in HS?

A
  • Tranfusion if needed (severe, symptomatic anemia)
  • Folic acid if moderate-severe anemia
  • Consider splenectomy and cholecystectomy in selected patients
32
Q

Typical features in HS (6)?

A
  1. Spherocytes
  2. Increased reticulocytosis
  3. Elevated RDW, elevated MCHC
  4. Coombs negative hemolytic process
  5. Increased osmotic fragility
  6. Positive family history
33
Q

Hereditary spherocytosis: inheritance, at risk populations

A

Inheritance: AD (75%); 25% with negative family history (likely de novo mutation)

At risk population: all ethnicities affected, especially prevalent in Northern European descent

34
Q

What is a Donath-Landsteiner antibody?

A

Cold reactiveIgG antibody that binds at 4C (in the extremities or face) and then activates complement when warmed at 37C

Usually directed against P antigen

35
Q

Name the differential diagnosis of acanthocytes?

A
  • Spurious
  • Hypothyroidism
  • Vitamin E deficiency
  • Anorexia nervosa
36
Q

What are the hematological manifestations of copper deficiency (2)?

A
  1. Microcytic anemia
  2. Neutropenia
37
Q

What form of iron is best absorbed?

A

Heme iron (present in meat) ­­­­> Fe2+ > Fe3+

Hematology (6 decks)

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