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Hematology Flashcards

(45 cards)

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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?

Study These Flashcards
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?

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

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

Study These Flashcards
A

hands/feet symmetrically (dactylitis)

22
Q

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

Study These Flashcards
A

acute chest syndrome

23
Q

How is acute chest syndrome defined?

Study These Flashcards
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?

Study These Flashcards
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
What kinds of bacterial infections are SCD patients highest risk for?
* encapsulated organisms (s. pneumoniae, n. meningitidis, h. influenzae) * salmonella (bacteremia, osteomyelitis) * patients should start PCN ppx once diagnosed
26
What is a common cause of aplastic crisis in SCD patients?
* 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
What is HbCC disease?
* Homozygous for HbC * mild hemolytic anemia, splenomegaly * no VOC issues
28
What is HbSC disease?
* equal amounts HbC and HbS * less anemic w/ less severe hemolysis than patients w/ HbSS
29
Describe G6PD disease
* **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
Describe Hereditary Spherocytosis
* **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
Warm AIHA vs. Cold Agglutinin Disease
* 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
How does aplastic anemia present, how is it diagnosed, what causes it, how is it treated, what are the types?
* 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
What is Fanconi Anemia (inheritance pattern, mechanism, clinic manifestations, diagnosis, treatment)?
* **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
What is Red Cell Aplasia and what are there different types?
* 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
How does Parvovirus B19 cause anemia, how does it present, what is the typical time course, and how to diagnose and treat?
* 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
What is Transient Erythroblastopenia of Childhood (TEC)?
* red cell aplasia * acquired, **self-limited** (resolves in 1-2 mo) * children **1-3 yo** * pallor + decreased activity * normocytic anemia + reticulocytopenia * treatment = **supportive**
37
What is Congenital Hypoplastic Anemia (Diamond-Blackfan), how does it present, how is it treated?
* 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
How to differentiate Pure Red Cell Aplasias?
* 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
How is severe neutropenia vs. mild neutropenia defined?
* Severe neutropenia: ANC _\<_ 500 * Mild neutropenia: ANC between 1000-1500
40
What are different etiologies of Neutropenia?
* **Inherited**: * cyclic neutropenia * severe congenital neutropenia (Kostmann syndrome) * Shwachman-Diamond syndrome * **Acquired**: * neonatal isoimmune neutropenia * chronic benign neutropenia (autoimmune) * virus- or drug- induced
41
What is Cyclic Neutropenia?
* 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
What is Severe Congenital Neutropenia (Kostmann Syndrome)?
* inherited neutropenia * rare, **AR** disorder * ANC typically \< 200 * also with **monocytosis** + **eosinophilia** * at risk for SBI and death * management: **G-CSF, BMT**
43
What is Shwachman-Diamond Syndrome?
* 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
What is Neonatal Isoimmune Neutropenia (NIN)?
* 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
What is Chronic Benign Neutropenia?
* 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

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