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Pediatric Boards > Hematology > Flashcards

Hematology Flashcards

1
Q

What kind of cell is this and what disease state is it associated with?

A

Burr cell

Disease: uremia

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2
Q

What kind of cell is this and what diseases is it associated with?

A

Spur cell

Disease: liver diseases

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3
Q

What kind of cell is this and what diseases is it associated with?

A

Target cell

Diseases: significant liver disease, thalassemia syndromes, sickle cell disease, homozygous hemoglobin C, other hemoglobinopathies

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4
Q

What kind of cell is this and what diseases is it associated with?

A

Sickle cell

Disease: sickle cell disease (HbSS), HbS beta-thalassemia; less common in HbSC

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5
Q

What kind of cell is this and what diseases is it associated with?

A

Teardrop cell (dacrocytes)

Disease: myelofibrosis and other infiltrating bone marrow processes, thalassemia

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6
Q

What kind of cell is this and what diseases is it associated with?

A

Elliptocyte

Disease: hereditary elliptocytosis, severe iron-deficiency anemia

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7
Q

What kind of cell is this and what diseases is it associated with?

A

Spherocyte

Disease: hereditary spherocytosis, autoimmune hemolytic anemia

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8
Q

What kind of cell is this and what diseases is it associated with?

A

Schistocyte

Disease: microangiopathic hemolytic anemia (TTP, HUS, HELLP syndrome, DIC, occasionally vasculitis), severe burns, valve hemolysis

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9
Q

What kind of cell is this and what diseases is it associated with?

A

Howell-Jolly Bodies (nuclear remnants)
Disease: splenectomy or functional asplenia

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10
Q

What kind of cell is this and what diseases is it associated with?

A

Basophilic Stippling (indicates ineffective erythropoeisis)

Disease: lead poisoning, thalassemia, pyrimidine 5’-nucleotidase deficiency

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11
Q

What kind of cell is this and what diseases is it associated with?

A

Hypersegmented polymorphonuclear leukocytes (neutrophils)

Disease: megaloblastic anemia (pernicious anemia/vit B12 deficiency, folate deficiency)

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12
Q

What are the three types of B-thalassemias and what kinds of Hb are associated with them?

A

B-thal minor

  • heterozygous - one normal and one thalassemia allele (either B+/B0)
  • mild anemia with lots of microcytes, asymptomatic
  • RDW nml
  • HbA2 > 3.5% is diagnostic

B-thal intermedia

  • homozygous (B+/B+)
  • some normal B-globin and some abnormal
  • mild sxs and no required transfusions
  • increased HbA2 and HbF

B-thal major

  • B0/B0 or B+/B0
  • essentially NO B-globin production
  • by 6-12 mo pallor, irritability, hepatosplenomegaly, sig anemia, jaundice, bone marrow expansion; transfusion dependent and often develop iron overload
  • HbF only
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13
Q

What are the 4 types of alpha-thalassemia?

A

the more loci affected, the worse the symptoms

  • a-thal trait: 1 locus, asymptomatic, no heme abnormalities (silent carrier)
  • a-thal minor: 2 loci, asymptomatic, low MCV, mild anemia
  • Hemoglobin H disease (HbH): 3 loci, moderate to severe hemolysis
  • hydrops fetalis: 4 loci, death in utero due to mainly Hb Bart (tetramer of gamma chains)
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14
Q

What is the most common cause of chronic GI blood loss worldwide?

A

Hookworm infection (Necator americanus or ancylostoma duodenale)

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15
Q

What is the most common cause of iron-deficiency anemia in pediatric population?

A

inadequate iron intake

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16
Q

How can you differentiate between iron-deficiency anemia and B/A-thalassemias?

A
  • RDW is normal in patients with thalassemias but increased in IDA patients
  • Mentzer Index: MCV/RDW
    • IDA: index > 13
    • thalassemia: index < 13
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17
Q

How can you differentiate iron-deficiency anemia vs. anemia of chronic disease?

A
  • Hepcidin = small protein released from hepatocytes that block Fe absorption in gut and iron release by hepatocytes and macrophages
    • IDA: low hepcidin levels (body wants to collect/release as much Fe as possible)
    • ACD: hepcidin nml
  • IDA: low Fe, high TIBC, low transferrin saturation, usually low ferritin
  • ACD: low Fe, low TIBC, low to nml transferrin sat, nml to high ferritin
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18
Q

What are the 2 types of pernicious anemia?

A

IF = intrinsic factor; produced by parietal cells of stomach

  • congenital pernicious anemia:
    • occurs <3 yrs old
    • consanguinity w/ AR inheritance
    • IF absent so no antibodies against it; otherwise nml gastric secretion
  • juvenile pernicious anemia
    • older children
    • autoimmune-mediated decrease in gastric IF
    • commonly assoc w/ gastric atrophy and decreased gastric fxn
    • commonly assoc with other AI conditions (i.e. vitiligo, thyroiditis)
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19
Q

What is the mutation that causes sickle cell disease?

A

point mutation on 6th codon of B-globin gene → adenine replaced by thymidine → valine encoded instead of glutamic acid → HbS polymerization and sickling

20
Q

What is the average lifespan of RBC in HbSS disease compared to normal?

A
  • SCD lifespan: 15-50 days
  • Normal lifespan: 120 days
21
Q

What part of body does the first SCD pain crisis typically involve?

A

hands/feet symmetrically (dactylitis)

22
Q

What is the leading cause of death in children and adults w/ SCD?

A

acute chest syndrome

23
Q

How is acute chest syndrome defined?

A

Development of new pulmonary infiltrate with one of the below:

  • fever
  • chest pain
  • tachypnea
  • hypoxia
24
Q

What is the typical treatment for acute chest syndrome?

A
  • antibiotics that cover pneumococcus, mycoplasma, and chlamydia (usually 3rd gen cephalosporin for routine bacterial cvg + macrolide such as azithromycin for atypical cvg)
  • respiratory support
  • hydration
25
Q

What kinds of bacterial infections are SCD patients highest risk for?

A
  • encapsulated organisms (s. pneumoniae, n. meningitidis, h. influenzae)
  • salmonella (bacteremia, osteomyelitis)
  • patients should start PCN ppx once diagnosed
26
Q

What is a common cause of aplastic crisis in SCD patients?

A
  • Parvo B19 infection
  • destroys early RBC precursors in bone marrow → BAD because SCD Hb lifespan only 15-50 days → can cause life-threatening anemia
  • low retic count
  • elevated Parvo IgM = acute infection
27
Q

What is HbCC disease?

A
  • Homozygous for HbC
  • mild hemolytic anemia, splenomegaly
  • no VOC issues
28
Q

What is HbSC disease?

A
  • equal amounts HbC and HbS
  • less anemic w/ less severe hemolysis than patients w/ HbSS
29
Q

Describe G6PD disease

A
  • X-lined recessive (Males > females)
  • reduced glutathione → less protection for RBC against oxidation stress
  • must avoid oxidant stress (fava beans, dapsone, primaquine, systemic infxn)
  • hemolytic crisis sxs = sudden onset pallor, fatigue, dark urine
  • peripheral smear = Heinz bodies (denatured globin), bite cells (macrophages removing heinz bodies)
  • cannot test for it during acute crisis b/c all messed up cells will be gone, must wait a couple months
30
Q

Describe Hereditary Spherocytosis

A
  • Autosomal Dominant (75%) or de novo mutation (10-25%)
  • structural or functional abnormality of cytoskeleton proteins (spectrin, ankyrin, band 3, protein 4.2)
  • normal shape RBC → goes through spleen and b/c cytoskeleton more rigid cannot easily pass thru → RBC membrane loss → cell becomes spherical and less deformable → increased risk for splenic lysis with each subsequent pass thru
  • diagnosis: anemia, reticulocytosis, increased MCHC, spherocytes on smear, positive osmotic fragility test
31
Q

Warm AIHA vs. Cold Agglutinin Disease

A
  • warm AIHA:
    • IgG Abs to Rh group antigen bind at body temp → macrophages and monocytes attack them → hemolysis
    • acute onset pallor, jaundice, dark urine
    • primary vs. secondary (infection, meds, lymphoproligerative and collagen vascular disorders)
    • positive direct Coombs
    • tx = steroids
  • cold disease
    • mycoplasma and EBV: IgM-RBC complexes activate complement → intravasc hemolysis
    • paroxysmal cold hemoglobinuria: keep patient warm and use blood warmer for transfusions; steroids NOT helpful
32
Q

How does aplastic anemia present, how is it diagnosed, what causes it, how is it treated, what are the types?

A
  • presents w/ pancytopenia
  • diagnosed via bone marrow exam (hypocellular or acellular bm)
  • cause: unknown in >50% cases; drugs (sulfa drugs), toxins (benzene, insecticides), infection, radiation
  • treatment for severe aplastic anemia: matched sibling stem cell transplant and immunosuppressive therapy
33
Q

What is Fanconi Anemia (inheritance pattern, mechanism, clinic manifestations, diagnosis, treatment)?

A
  • Autosomal Recessive
  • Poor DNA repair mechanisms
  • short stature, skeletal abnormalities (absent or abnml thumbs, abnml radii, microcephaly, vertebral abnml), cafe au lait spots, hyperpigmentation or hypopigmentation, renal anomalies
  • diagnosis: identification of DNA repair issues
  • 40-50% risk of myelodysplastic syndrome, 15% risk of AML by age 50, increased risk of hepatic malignancy and squamous cell carcinoma
  • cure: HSCT; however does NOT decrease risk for cancers
  • FANCONI SYNDROME IS A DIFFERENT PROBLEM
34
Q

What is Red Cell Aplasia and what are there different types?

A
  • red cell aplasia = anemia in setting of reticulocytopenia
  • Types:
    • Parvovirus (B19)
    • transient erythroblastopenia of childhood (TEC)
    • congenital hypoplastic anemia (Diamond-Blackfan)
  • can also have idiopathic cause or 2/2 drugs or immune disorders
35
Q

How does Parvovirus B19 cause anemia, how does it present, what is the typical time course, and how to diagnose and treat?

A
  • Parvovirus infects erythroid progenitors → acute or chronic red cell aplasia
  • red cell aplasia + fever, rash (slapped cheek), and/or arthropathy
  • Time course:
    • in healthy patient is transient
    • in immunocompromised patients can become chronic
    • in SCD patients can cause aplastic crisis
  • Diagnosis: Parvovirus B19 DNA and/or IgM
  • treatment: supportive in healthy patients, IVIG in complicated cases among immunocompromised patients
36
Q

What is Transient Erythroblastopenia of Childhood (TEC)?

A
  • red cell aplasia
  • acquired, self-limited (resolves in 1-2 mo)
  • children 1-3 yo
  • pallor + decreased activity
  • normocytic anemia + reticulocytopenia
  • treatment = supportive
37
Q

What is Congenital Hypoplastic Anemia (Diamond-Blackfan), how does it present, how is it treated?

A
  • red cell aplasia
  • macrocytic anemia + reticulocytopenia
  • diagnosed < 1 yo
  • presentation: thumb anomalies, dysmorphic features (snub nose, thickened upper lip, wide-set eyes), short stature, glaucoma, renal anomalies, hypogonadism, short webbed neck, congenital heart disease, intellectual disability
  • management: transfuse until 6 mo - 1 yo → steroids after 12 mo (not earlier because of bone growth)
    • anemia responds to steroids in 80% of cases, spontaneous remission in 25%
    • if steroid refractory or dependent → chronic transfusions and possibly stem cell transplant
38
Q

How to differentiate Pure Red Cell Aplasias?

A
  • anemia + low reticulocytes = red cell aplasia (Parvo B19, TEC, Diamond Blackfan)
  • Parvo: school-aged children, associated fever/rash/arthropathy
  • TEC: children between 1-4 yo, normocytic RBCs, no fever/rash/arthropathy
  • DBA: usually diagnosed < 1 yo, macrocytic RBCs, congenital deformities
39
Q

How is severe neutropenia vs. mild neutropenia defined?

A
  • Severe neutropenia: ANC < 500
  • Mild neutropenia: ANC between 1000-1500
40
Q

What are different etiologies of Neutropenia?

A
  • Inherited:
    • cyclic neutropenia
    • severe congenital neutropenia (Kostmann syndrome)
    • Shwachman-Diamond syndrome
  • Acquired:
    • neonatal isoimmune neutropenia
    • chronic benign neutropenia (autoimmune)
    • virus- or drug- induced
41
Q

What is Cyclic Neutropenia?

A
  • inherited neutropenia
  • neutropenia occurs at regular interval (every 21 +/- 3d)
  • defective maturation of uncommitted stem cells
  • during neutropenic period: fever, aphthous stomatitis, pharyngitis, cervical lymphadenitis, rectal/vaginal ulcers
  • at risk for sepsis caused by clostridium septicum
  • ⅓ of patients get it via AD inheritance
  • management: G-CSF and antibiotics, excellent oral hygiene
42
Q

What is Severe Congenital Neutropenia (Kostmann Syndrome)?

A
  • inherited neutropenia
  • rare, AR disorder
  • ANC typically < 200
  • also with monocytosis + eosinophilia
  • at risk for SBI and death
  • management: G-CSF, BMT
43
Q

What is Shwachman-Diamond Syndrome?

A
  • inherited neutropenia
  • AR inheritance mutation in SBDS gene
  • present similar to CF patients: FTT, steatorrhea due to pancreatic exocrine deficiency, recurrent infections
    • NORMAL SWEAT TEST
  • diagnostic eval: CBC, BM aspirate and biopsy, serum isoamylase, serum pancreatic trypsinogen, fecal elastase levels, SBDS gene analysis
  • treatment: supportive w/ pancreatic enzyme replacement, G-CSF, BMT
  • increased risk for AML and myelodysplastic syndrome
44
Q

What is Neonatal Isoimmune Neutropenia (NIN)?

A
  • acquired neutropenia
  • self-limited disease that occurs in newborns
  • similar to Rh disease (except mother sensitized to fetal neutrophil antigens of paternal origin)
    • mother does not have these antigens →produce maternal Abs → IgG Abs cross placenta and destroy fetal neutrophils → neutropenia
  • ANC recovers in 6-12 weeks
  • subsequent siblings at risk as well
45
Q

What is Chronic Benign Neutropenia?

A
  • acquired neutropenia
  • also known as autoimmune neutropenia
  • ANC persistently < 1000 due to autoantibodies to granulocytes
  • most common kind of neutropenia in healthy kids
  • AD inheritance or sporadic
  • median diagnosis age 8-11 mo; resolves after ~ 2 yrs
  • usually mild; if severe give G-CSF

Pediatric Boards (5 decks)

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