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PedsSpring2020 > Case 30: 2yo - Sickle cell disease, hemolytic anemia > Flashcards

Case 30: 2yo - Sickle cell disease, hemolytic anemia Flashcards

1
Q

4yo child previously healthy (no meds), recently came back 2w ago from Pakistan –> fevers (up to 102F), chills for 3d, very tired over last 24h.
Hgb 8.5
WBC 8.2
Plt 410

  • Had been dehyrated, given fluid bolus
  • Given Tylenol for the fevers
  • Mildly jaundiced (scleral icterus)
  • brown urine every time he has a fever (tea colored): chills - fever - brown urine ==> then clears until next fever.

Negative Fhx

Vitals unremarkable

Physical exam - no rash, hepatosplenomegaly, abd masses, no fixed nodes

what tests do you want next?

A
  • CBC = Hgb, WBC, Plt, MCV
  • Reticulocyte count («1% production problem v. &raquo_space;1% destruction problem = hemolysis) ==> can falsely elevate MCV
  • ferritin (can be elevated with illness)
  • haptoglobin
  • peripheral blood smear
  • UA
  • Direct Coombs (DAT) = to determine if autoimmune or not
  • Hgb electrophoresis (if DAT negative)

==> ultimately DAT+ for C3 (no IgG)
+send out for Donath landsteiner test
paroxysmal cold hemoglobinuria

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2
Q

MCV lower limit of normal

A

70+age

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3
Q

Anemia in a kid with URI. is this normal?

A

not “normal” – but likely due to viral cause of destruction of >/=1 cell line

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4
Q

4yo with fever, anemia, and rash

A

Diffdx: parvo – b/c everyone destroys RBCs with this

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5
Q

in a kid with iron deficiency anemia, what is the first sign?

A

1) increased RDW >16%

2) decrease in Hgb, MCV

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6
Q

autoimmune hemolytic anemia labs

A
  • CBC = low Hgb, nml WBC, high Plt, nml MCV
  • Reticulocyte count&raquo_space;1% destruction problem = hemolysis

-ferritin (can be elevated with illness)
- haptoglobin <31 (low d/t loss increasing binding of iron ==> hemolysis)
-peripheral blood smear = spherocytes, helmet cells / schistocytes
-UA = 1.03 / pH 65 / 2+ heme / no protein, no RBCs, no WBCs
==> intravascular hemolysis (b/c + heme, - RBCs) rather than renal issue
-Direct Coombs (DAT): 3+ == positive; IgG or C3

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7
Q

diffdx:

non-autoimmune hemolytic anemia

A

1) hemoglobinopathy (congenital hemolytic anemia)
2) membranopathy == hereditary spherocytosis, hereditary ellipticocytosis
3) RBC enzyme deficiency == G6PD deficiency, pyruvate kinase deficiency

4) paroxysmal nocturnal hemolytic anemia = membrane deficiency in CD55/CD59, with hyper-attachment of C5 (complement)

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8
Q

TTP

A

Adam13 mutation == cleaves high

congenital == does not make enzyme Adam13
autoimmune == make antibody against Adam13

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9
Q

diffdx for spontaneous thromboses

A

paroxysmal nocturnal hemolytic anemia

Factor 5 lyden

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10
Q

diffdx:

autoimmune hemolytic anemia (positive Coombs)

A

IgG +/- C3 == warm auto-Ab

C3 +, IgG -

1) cold agglutinin (IgM+, cold agglutinin titer HIGH) == hemolyze at cold temperatures. Attaches to antigen “I”
2) paroxysmal cold hemoglobinuria (IgG+ in cold environment [periphery body], then hemolyze in warm environment [central body]. Attaches to antigen “P” ==> Fever (with cold extremities = IgG+ in extremities) –> the spread throughout ==> HEMOLYZE

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11
Q

how to treat warm v. cold agglutinin hemolytic anemia

A

warm agglutinin

1) cross-match ==> find the least incompatible unit of blood
2) Steroids

cold agglutinin - either cold / paroxysmal cold ==> want to keep warm so that they don’t hemolyze
1) KEEP WARM - no cold fluids, foods
2) TRANFUSE with WARM BLOOD
+/- steroids in PCH

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12
Q

conditions associated with cold agglutinins

A

EBV
Mycoplasma
Hep C

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13
Q

how to diagnose paroxysmal cold hemoglobinuria

A

Donath Landsteinertest = serum (with IgG) + non-patient RBCs at 4 C –> 37C

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14
Q

what hereditary blood disorders protect against Malaria

A

G6PD deficiency
sickle cell disease/trait
thalassemia

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15
Q

define: malaria belt

A

mediterranean == beta thalassemia
Middle East == beta thalassemia
Africa
south east asia == alpha thalassemia

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16
Q

what can precipitate paroxysmal cold hemoglobinuria?

A

Viral illness ==> causing fever ==> causing MASSIVE hemolysis over his baseline level of hemolysis ==> tea- or brown-colored urine

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17
Q

complications of hemolytic anemia

+/- giving more blood in autoimmune hemolytic anemia

A

Fulminant real failure

prevention

  • fluid boluses
  • 2* MIVF
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18
Q

reticulocyte production index (RPI)

A

reticulocyte production index (RPI)

RPI = retic% * (pt Hct / nml Hct)

RPI = retic% * (pt Hct / 45)

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19
Q

sickle cell disease (SCD)

  • pathophysiology:
  • signs and symptoms
  • labs / findings
A
  • pathophysiology [AUTOSOMAL RECESSIVE] substitution of valine FOR glutamic acid in Hgb molecule ==> formation of Hgb polymers that becomes deoxycgenated ==> “sickling” of RBCs
    ==> increased adherence, block blood flow in microvasculature ==> local tissue hypoxia, pain, tissue damage
    ==> induced hemolysis of RBCs ==> chronic anemia ==> increased reticulocyte count
  • signs and sxs
  • anemia
  • jaundice = increased breakdown of RBCs
  • pain and swelling in extremities
  • fatigue, need to sleep more
    1) splenic enlargement (@2-3yo), then splenic fibrosis (@4-5yo)
    2) jaundice == d/t RBC hemolysis
    3) signs of stroke
  • CBC: leukocytosis, thrombocytosis @ stress
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20
Q

describe the Hgb type ad what the pattern means:

F

A

predominant hgb at birth

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21
Q

describe the Hgb type ad what the pattern means:

A

A

normal adult hemoglobin

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22
Q

describe the Hgb type ad what the pattern means:

FAS

A

Baby carries one normal, one abnormal (S) allele

==> benign sickle cell trait

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23
Q

describe the Hgb type ad what the pattern means:

FAC

A

Baby carries one normal, one abnormal (S) allele

==> benign Hgb C trait

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24
Q

describe the Hgb type ad what the pattern means:

FS

A

BOTH alleles have mutation for Hgb S

==> sickle cell disease
- splenomegaly in childhood; splenic fibrosis in teens

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25
Q

describe the Hgb type ad what the pattern means:

FSA

A

sickle cell beta thalassemia
1 globin gene has mutation for S
other globin gene has mutation for B-thalassemia == no Hgb or dysfunctional Hgb

==> sickling disorder, milder v. sickle cell disease
(treated similarly to sickle cell disease - FS)
- can have splenomegaly into teens

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26
Q

describe the Hgb type ad what the pattern means:

FA

A

normal

no sickling disorder

27
Q

describe the Hgb type ad what the pattern means:

FSC

A

sickle cell Hgb C
1 globin gene has mutation for Hgb S
other globin gene has mutation for Hgb C

==> sickling disorder, milder v. sickle cell disease
(treated similarly to sickle cell disease - FS)
- can have splenomegaly into teens

28
Q

You know that sickle cell disease (FS) gets treated a certain medications. what two other diseases that are related, are treated similarly?

A

FSA = sickle cell beta thalassemia

FSC = sickle cell hemoglobin C disease

29
Q

common procedures for young individuals with sickle cell disease

A

1) tonsillectomy +/- adenoidectomy for improved OSA

2) Cholecystectomy for symptomatic cholelithiasis (even first gallstone)+ preoperative pRBC transfusion to reduce changes of acute chest syndrome d/t anaesthesia time
+ treatment for cholecystitis

3) splenectomy == for splenic sequestration crisis (fatigue, SOB)

30
Q

complications of sickle cell disease

A
  • febrile illnesses
  • vasoocclusive problems
  • growth delay == d/t chronic anemia, poor nutrition, painful crises, endocrine dysfunction, poor pulmonary fx

1) lymphoidal tonsillar tissue hypertrophy involving Waldeyer’s ring –> excessive snoring, OSA, ? increased risk of sickling due to tonsillar hypertrophy and desaturation of Hgb
2) bilirubin gallstones (in all hemolytic anemias - by adolescent years) ==> d/t increased release of Hg during breakdown of abnormal RBCs
3) sepsis (esp in infants, young children) ==> d/t decreased splenic function, risk for encapsulated organisms (Strep pneumo, H. flu B, Neisseria meningitidis
4) RBC aplasia (more severe anemia) == d/t myelosuppression from parvovirus/hypersplenism (spleen enlarges, traps RBCs)

5) stroke == 10% by 15yo
(need transcranial Doppler)

6) respiratory problems d/t vaso-occlusive sxs
==> pneumonia - increased risk of infection
==> acute chest syndrome == vaso-occlusion in lung parenchyma (tx = supplemental O2, transfusion therapy, morphine)
==> rib infarction
+/- chronic lung disease

7) sickle cell splenic sequestration crisis @ 1-3yo == when blood pools in the spleen –> splenomegaly, pallor, fatigue, irritability ==> anemia, shock
8) splenic fibrosis @ 4-6yo

9) cardiomegaly at baseline
+/- CHF ==> d/t high output strain

10) sepsis
11) sickle retinopathy
11) aseptic necrosis of femoral heads
12) sickle cell nephropathy

31
Q

prophylaxis needed for sickle cell disease

A

1) Penicillin yearly until 5-6yo –> decreased risk of sepsis

IMMUNIZATIONS
==> for kids <2yo == conjugation vaccines are better (v. purely polysaccharide vaccine) to increase chance that it will make antibodies
1) pneumococcal vaccine (13-valent conjugate) @ 2, 4, 6mo
2) pneumococcal vaccine (23-valent polysaccharide) @ 2,5yo

3) H. flu type b conjugate @ 2, 4, 6mo

4) meningococcal conjugate @ 2yo
+ booster @ 5-7yo.

32
Q

why is fever so scary in kids with sickle cell disease?

what is the evaluation?

A
  • fever may be the only sign of sepsis in children with sickle cell disease ==> EMERGENCY
    b/c lack normal splenic function, and subtle immunologic deficiencies
    1) blood cultures + broad-spectrum IV antibiotics
    2) D/C only after 48-72h, if cultures negative, patient is well
33
Q

goals for comprehensive visit for SCD

A
  • how family deals with various complications
  • frequency of painful or other vaso-occlusive problems
  • how the family accesses health care
  • how family is dealing with a chronic illness
34
Q

treatment for sickle cell disease

A

ACUTE vaso-occlusive sxs

  • oxygenation
  • RBC transfusions
  • morphine

CHRONIC

  • BID penicillin ==> To prevent infections that can lead to sepsis
  • hydroxyurea = with frequent painful / vaso-occlusive problems ==> decrease severity and frequency of sickling disorders == improved NO metabolism, reduced RC endothelial interaction, decreased RBC density, increased total HbF

+/- stem cell transplantation == (1) myeloblative therapy (major cause of death), (2) then hematopoetic stem cell transplant (or bone marrow transplate)

35
Q

effect of chronic illness on growth and development

A
  • poor academics d/t frequent absences
  • decreased socialization and self esteem
  • financial hardship
  • sibling neglect
  • guilt in siblings b/c they aren’t the ones whoare ill
36
Q

what are some prenatal tests for genetic diseases?

A
  • fetal blood in utero

- chorioamnionitis

37
Q

baseline Hgb in sickle cell disease

A

Hgb 6-9
usually okay without problems

–> problems when any acute change

38
Q

acute chest syndrome in sickle cell disease

  • define/signs and sxs
  • causes:
  • labs
  • management
  • treatment
A

-define: cough and SOB
+ low SaO2, fever, cough, chest pain

  • causes/triggers: pneumonia (viruses, chlamydia, mycoplasma, bacteria), intrapulmonary sickling, pulmonary fat embolism / infarction –> atelectasis
  • leukocytosis, thrombocytosis (d/t stress)
  • anemia, increased reticulocyte count (as BM rallies)
  • management: workup for infection, pulmonary/respiratory complaints; pain control (monitor for respiratory depression)
  • treatment = O2 + MIVF+ IV NSAIDS + IV narcotics (PCA)
  • not oral b/c respiratory distress and tachypnea
  • s/e = pulmonary edema from fluid overload
    +/- antibiotics for pneumonia
39
Q

describe the CXR findings in: Acute chest syndrome

A

==> d/t fat embolism, vaso-occlusion of pulmonary vasculature

  • multilobar infiltrates (esp. LL and ML)
  • effusions
  • atelectasis
  • cardiomegaly (for sickle cell disease at baseline)
40
Q

describe the CXR findings in: pericarditis

A

of the heart

  • effusion
  • infiltrate
41
Q

describe the CXR findings in: CHF

A
  • LL infiltrates

- cardiomegaly

42
Q

describe the CXR findings in: rib infarction

A
  • pleural effusion

NO pulmonary infiltrates
NO cardiomegaly
NO hypoxia

43
Q

describe the CXR findings in: sepsis

A
  • cardiomegaly == if in cardiogenic shock

- ARDS == new infiltrates

44
Q

considerations in treatment of pain (in sickle cell disease)

A
  • problem of pain in sickle cell disease (or in post-surgery, rib fractures) == decreases ability to fully expand lungs
    ==> worsen pulmonary status
    ==> increased risk of pneumonia

1) narcotic analgesia –>s/e = central respiratory depressant
2) non-narcotic analgesia == NSAIDS == analgesic + anti-inflammatory

45
Q

considerations in treatment of atelectasis (in sickle cell disease)

A

atelectasis (= collapsed / airless segment of lung d/t obstruction + distal collapse of alveoli)
==> can exacerbate acute chest syndrome ==> can be life-threatening

tx = encourage deep breathing (incentive spirometer = increased inspiratory effort = decreased atelectasis), walking

46
Q

indications for RBC transfusion in acute chest syndrome

  • what else needs to be done at that time?
A

1+ of:

  • decrease in Hgb from baseline (again, 6-8)
  • increasing RR
  • worsening chest syndrome
  • declining O2 sats (<88-90%)
  • progressive infiltrates on CXR
Other studies (PRIOR TO TRANSFUSION)
- complete RBC antigen phenotype of his own RBCs ==> to phenotypically match blood for pt's specific antigens
==> decrease risk of alloimminzation to minor antigens
47
Q

indications for erythrocytapheresis

A

== exchange transfusion = rapidly lower the hemoglobin S level and rapidly raise the hemoglobin

1+ of:

  • severe sickle cell disease
  • hypoxemia not corrected by O2 therapy
  • failing pulmonary function
  • acute onset of stroke ==> to lower HgS level to help prevent progression and prevent recurrent stroke
48
Q

diffdx for fever, respiratory distress, chest pain in child with sickle cell disease

A

ACUTE CHEST SYNDROME==> d/t painful vaso-occlusive crisis
- fever, cough, chest pain (+/- atelectasis d/t difficulty with expansion of LL), SOB, decreased SaO2
+ splenomegaly

PERICARDITIS
- tachypnea, fever, +/- chest pain

CHF ==> can be due to sickle cell disease, chronic anemia
- tachypnea
+ NO chest pain

RIB INFARCTION ==> d/t sickle cell disease
- fever, tachypnea (with shallow breaths d/t pain)

SEPSIS ==> d/t sickle cell disease

49
Q

most common presentation of CHF in children

A

tachypnea

50
Q

most common presentation of CHF in children

A

tachypnea

51
Q

indications for emergency treatment of sickle cell disease

A
  • fever ==> may be only sign of serious disease
  • splenic enlargement (increased size / rapid increase) ==> splenic sequestration crisis
  • slurred speech ==> stroke
  • chest pain ==> acute chest syndrome
  • tachypnea ==> acute chest syndrome
  • increased pallor (over baseline) ==> splenic sequestration, increased hemolysis, temporary inhibition of erythroid production (aplastic crisis - Parvo B19)
  • increased jaundice (over baseline) - increased hemolysis / need for transfusion (+/- viral illness)
  • priapism ==> d/t sickling in penile arteries ==> permanent damage
52
Q

are antibiotics indicated in kids with acute chest syndrome?

A

UNSURE
- b/c clinical and laboratory features of ACS do not allow one to easily
distinguish whether the cause is pulmonary fat embolism and/or infarction from acute infectious
pneumonitis.

53
Q

Which of the following hemoglobin patterns are associated with a diagnosis of sickling disorders on the newborn screen? Select all that apply.

 Multiple Choice Answer:
A 	FA
B 	FS
C 	FAS
D 	FSA
E 	FSC
F 	FAC
A 
FS = sickle cell disease 
FSA = sickle cell beta thalassemia
FSC = sickle cell Hgb C
54
Q

Gerardo’s chart shows that he has never had surgery. Which of the following are the two most common surgeries in children with sickle cell disease? (Select all that apply.)

 Multiple Choice Answer:
A		Tonsillectomy	
B		Herniorrhaphy	
C		Appendectomy	
D		Tympanostomy	
E		Cholecystectomy
A

Tonsillectomy == b/c can become enlarged and cause OSA

Cholecystectomy == d/t bilirubin gallstones from hemolysis

55
Q

when is transcranial Doppler study indicated in kids with sickle cell disease

A

children with sickle cell disease @ 2-15yo
==> to predict risk of patients for stroke via evaluation of cerebral blood-vessel flow
==> to evaluate which patients might benefit from prophylactic transfusion of RBCs

56
Q

Which immunization(s) should be given today at the 2yo visit for a kiddo with sickle cell? (Select ALL correct answers.)

 Multiple Choice Answer:
A		Meningococcal conjugate	
B		Diphtheria, tetanus, pertussis	
C		Haemophilus influenzae type b	
D		23-valent pneumococcal polysaccharide vaccine (pneumococcal polysaccharide vaccine)	
E		Measles, mumps, rubella
A

A, D

DTAP is not as important for sickle cell kids

pneumococcal @ 2yo == 23 valent polysaccharide

Hib @ 2, 4, 6mo == already completed prior to this

57
Q

important parts of the physical exam to focus on in a kid with sickle cell

A
  • splenic side (large / small)
  • sclera == jaundice
  • neurologic exam == stroke
58
Q

when seeing a kid with sickle cell disease, which of the following pieces of info do you need to correctly interpret the CBC?

 Multiple Choice Answer:
A 	History of transfusions
B 	Several recent CBCs (baseline values)
C 	Erythrocyte sedimentation rate
D 	platelet count
E 	Frequency of pain crises
A

A, B

== just pure numbers

59
Q

Aplastic crisis

  • define:
  • cause:
  • why is this more dangerous for kids with sickle cell disease?
A
  • define: temporary inhibition of erythroid production frequently associated with viral illness
  • cause: ParvoB12
  • more dangerous for kids with sickle cell disease: BASELINE rapid destruction of RBCs and lower Hct + BRIEF INHIBITION of RBC production d/t viral infection

==> concerns about significant anemia.

60
Q

how to assess pain in a 2yo

A
  • faces pain scale

- parental assessment

61
Q

A 5-year-old African-American male with sickle cell disease presents to clinic with a chief complaint of severe chest pain for the past day. His mother notes that he has been breathing quickly and that she measured his temperature this morning to be 100.5 F. Patient describes pain as an 8/10 on the faces scale. Patient is tachypneic on exam and has an oxygen saturation of 97% on room air. Chest exam reveals normal lung sounds bilaterally, and he has some reproducible tenderness over his chest wall. A chest x-ray is performed and demonstrates clear lung fields and a cardiac silhouette that is within normal limits. What is the most likely cause of the chest pain?

 Single Choice Answer:
Please select one answer.  
A		Acute chest syndrome	
B		Rib infarction	
C		Sepsis	
D		CHF	
E		Pneumothorax
A

B

still a fever

all else is clear

always have to think of rib infarction with sickle cell

Rib infarction is correct because of the chest pain in the setting of a history of sickle cell disease with normal oxygen saturation and clear lung fields on chest x-ray. Osteomyelitis and painful vaso-occlusive crises represent the most common reasons for admission for sickle cell disease patients. Rib infarction may lead to a picture similar to ACS as the pain can lead to hypoventilation, which may result in atelectasis and the characteristic radiographic findings of ACS. However, no radiographic findings are present in this patient. As a result, rib infarction is the most likely etiology.

Acute chest syndrome (ACS) is incorrect, because there is normal oxygen saturation, and normal lung fields on radiological investigation. Lung markings on chest x-ray suggestive of ACS would include pulmonary infiltrates, atelectasis, and effusion. Causes of ACS include infection, pulmonary fat embolism, or intrapulmonary sickling.

62
Q

A 4-year-old patient with sickle cell disease presents for a well child evaluation. She has a history of three sickle cell vaso-occlusive crises in the past, including dactylitis and bone pain. She has been symptom-free for a few months and today she is feeling well. She is meeting her developmental milestones. She had an upper respiratory infection recently, but seems to be getting better now. She is up to date on her standard vaccinations up to 2 years including a full course (four doses) of Prevnar. What would you do for her today?

Single Choice Answer:
Please select one answer.
A Echocardiogram and transcranial ultrasound
B Quantiferon gold test
C HPV vaccine
D A dose of pneumococcal polysaccharide vaccine (Pneumovax).
E Chest x-ray

A

D

Prevnar = 13-valent conjugate

but still need the 23-valent polysaccharide @ 2yo

All children should routinely receive the Prevnar vaccine series. Patients with risk factors for pneumococcal sepsis, such as those with sickle cell disease, damaged spleen or asplenia, cochlear implants, CSF leaks, HIV, immunocompromise, chronic hear/lung disease, or those taking immunosuppressive medication should also receive the 23-valent polysaccharide pneumococcal vaccine at age 2 years.

Echocardiogram and transcranial ultrasound are recommended for patients with sickle cell disease. Echocardiogram screens for evidence of left ventricular hypertrophy and cardiomyopathy. Transcranial ultrasound is used to evaluate risk of stroke by measuring the middle cerebral artery flow. Routine transcranial ultrasound screening for these patients is recommended; echocardiogram is usually recommended for patient older than 21 years old, or for those with cardiopulmonary symptoms.

63
Q

You are notified that a 10-day-old patient in your practice had a newborn hemoglobin screen positive for sickle cell disease. Pregnancy and delivery were uncomplicated. Mother is 19 years old and works as a nurses’ assistant at a nursing home. When questioned, she says she remembers her grandmother died of chest pain and a lung infection. Which of the following should be ordered next for the baby?

 Single Choice Answer:
Please select one answer.  
A		Antibiotics	
B		CBC	
C		Chest x-ray	
D		H. influenza type b vaccination	
E		Transcranial Doppler
A

A

penicillin at birth

This newborn has sickle cell disease. Her grandmother likely passed away from acute chest syndrome, a common complication of this disease. Due to decreased splenic function and consequent decreased resistance to infection with encapsulated organisms (Streptococcus pneumoniae, Haemophilus influenzae type b, Neisseria meningitidis), infants and young children with sickle cell disease are at increased risk for sepsis. When given to infants with sickling disorders, penicillin significantly decreases the risk of mortality from overwhelming sepsis. Prophylaxis is usually continued until the child is five or six years of age (after this, there is little data to support its use except in patients who have had documented sepsis and bacteremia, or who have had their spleens removed).

all other vaccinations (except Hep B) indicated @ 2, 4, 6mo

64
Q

Devin is a 2-year-old African-American boy with sickle cell disease. Today his parents brought him to the ED because he was not feeling well. His mother reports that he has been very tired for the past week. Vitals show a temperature of 39°C, BP of 120/75 mmHg, RR of 24 bpm, and HR of 104 bpm. On physical examination he is ill appearing. His conjunctivae appear pale, and his sclerae are anicteric. Lungs are clear to auscultation. His abdominal exam is benign (non-tender, non-distended, with no organomegaly). His extremities are non-tender upon palpation. His nailbeds appear pale. Stat CBC reveals Hgb: 4.5 g/dL, Hct 15%, WBC 1800, and platelets 88,000. Mother is concerned as she has never seen him so ill before. What is the most likely cause of his new symptoms?

 Single Choice Answer:
Please select one answer.  
A		Vaso-occlusive crisis	
B		Splenic sequestration	
C		Aplastic anemia	
D		Hemolytic crisis	
E		Chest crisis
A

C

fever
?mild tachypnea, tachycardia
pallor
NO PAIN
NO SPLENOMEGALY
NO JAUNDICE
LITTLE PULMONARY SXS

extreme anemia, more so than usual. something going after the RBCs

Parvovirus B19 causes aplastic anemia, especially in children with shortened RBC half-life. A mild anemia associated with parvovirus often goes unnoticed in healthy children. However, in patients with decreased RBC survival, such as sickle-cell patients, anemia can develop very rapidly and severely, along with decreases in the other cell lines (resulting in leukopenia and thrombocytopenia). In the above presentation, the presence of fever in a child with sickle cell disease should also raise concern for significant bacterial infection from encapsulated organisms like pneumococcus. This patient should have a blood culture and be started on empiric antimicrobial therapy pending screen labs and cultures.

PedsSpring2020 (37 decks)

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