Table 1: Causes of Functional Asplenia 

 

Congenital

            Asplenia

            Cyanotic heart disease

 

Hematological

            Sickle-cell disorders

            Essential thrombocytopenia

            Hodgkin’s lymphoma

            Non-Hodgkin’s lymphoma

            Fanconi’s syndrome

 

Autoimmune

            Systemic lupus erythematosus

            Mixed connective tissue disease

            Rheumatoid arthritis

            Sjögren’s syndrome

 

Gastrointestinal

            Celiac disease

            Ulcerative colitis

            Crohn’s disease

            Tropical sprue

            Whipple’s disease

            Alcoholic liver disease

 

Circulatory compromise of the spleen

 

Miscellaneous

            Irradiation

            Amyloidosis

            Sarcoidosis

            AIDS

            Allogeneic Bone Marrow Transplantation

            Long-term intravenous nutrition

            Selective IgA deficiency

 

Table 2:  Pathogens associated with OPSI

 

Definite

Streptococcus pneumoniae

Capnocytophaga canis (Dysgonic fermenter 2 [DF2])

Bordetella holmesii

Babesia microtis (cattle malaria)

Plasmodia spp.  (malaria)

 

Possible

Haemophilus influenzae type b (Hib)

Salmonella species

 

Unlikely

Neisseria meningitidis

 

Table 3: Differential Diagnosis

 

Sepsis (infection) with severe sepsis/septic shock

Painful crisis (sickle cell disease)

Acute chest pain syndrome (sickle cell disease)

Pneumonia (without severe sepsis/septic shock)

Pulmonary embolism

Malignancies (lymphoma, leukemia and others)

Infarction of lung and other cardiopulmonary compromise

Collagen-vascular disorders

Drug-associated fever

 

Table 4: Clinical Manifestations

Fever with rigors

Arthralgias and myalgias

Headache

Gastrointestinal symptoms (upper and lower GI tracts)

Rash

Cardiovascular collapse

Disseminated intravascular coagulopathy

Seizures

Coma

Gangrene (Purpura fulminans)

 

Table 5: Laboratory and Radiological Investigations

 

Routine

 

 

 

 

 

Complete chemistry profile

 

 

Complete blood count and differential

 

 

Coagulation assays

 

 

Arterial blood gases

 

 

Peripheral smear

 

 

Fibrin, fibrin split products

 

 

Cortisol level

Cosyntropin stimulation test, if required

 

Blood cultures

At least two sets, two separate sites

 

Urinalysis

Urine culture, if indicated

 

Sputum specimen

Culture and Gram’s stain

 

Cerebrospinal fluid

Cell count, protein, glucose

Gram’s stain, culture

S. pneumoniae urine antigen

 

 

 

 

 

Specialized

 

 

 

 

 

Buffy coat

if neutropenic

 

Plasmodia spp.   

Thin blood smear

ParaSight F and OptiMal rapid assays

Babesia

Thin blood smear

IgG IFA assay (CDC)

 

 

 

Radiology

 

 

 

 

 

Chest radiography

 

 

Ultrasonography

As indicated by clinical or laboratory findings

 

CT and MRI scans

As indicated by clinical or laboratory findings

 

Nuclear scans

As indicated by clinical or laboratory findings

 

 

Table 6a: Empiric Antibiotic Therapy, Adults

Antibiotic (trade)

Usual dosage

Meningitis dosage

Renal adjustment

 

Cefotaxime (Claforan)

1-2 gr IV every 8 hrs

2 gr IV every 4-6 hrs

Increase interval to 12-24 hrs

Ceftriaxone (Rocephin)

 

1 gr IV once daily

2 gr IV every 12 hrs

Not applicable

Vancomycin

 

 

 

1 gr IV every 12 hrs,

Pharmacokinetics recommended

Same; pharmacokinetics recommended

Reduction required; antibiotic levels require monitoring

Ciprofloxacin (Cipro)

400 mg IV every 12 hrs or 500-750 mg PO every 12 hrs

 

400 mg IV every 12 hrs or 750 mg PO every 12 hrs

50% reduction

Levofloxacin (Levaquin)

750 mg IV/PO daily

Same

750 mg IV/PO once, then 500 mg IV/PO every 48 hrs

 

Moxifloxacin (Avelox)

400 mg IV/PO every 24 hours

Same

Clinical data lacking for CNS infections

 

 

 

 

 

Imipenem (Primaxin)

500 mg IV every 6 hrs

Same

125-250 mg IV every 12 hrs; beware of inducing seizures if dosage is exceeded

 

Meropenem (Merrem)

1 gr IV every 8 hrs

Same

0.5-1 gr IV every 12-24 hrs

 

Table 6b: Empiric Antibiotics Therapy, Pediatric

 

Antibiotic (trade name)

Usual dosage

Meningitis dosage

 

Cefotaxime (Claforan)

50 mg/kg IV every 8 hrs

75 mg/kg IV every 6 hrs

 

Ceftriaxone (Rocephin)

50 mg/kg IV every 24 hrs

100 mg/kg IV every 24 hrs

 

Imipenem (Primaxin)

15-25 mg/kg every 6 hrs (maximum dosage 2-4 gr/day)

 

Not recommended due to seizure potential

Meropenem (Merrem)

60-120 mg/kg IV divided every 8 hrs

120 mg/kg IV divided every 8 hrs

 

Rifampin (Rifadin)

10 mg/kg PO/IV daily

Same

Vancomycin

40 mg/kg IV divided every 6-8 hrs

60 mg/kg IV divided every 6-8 hrs

 

Fluoroquinolones

not approved for this population, in this setting

 not approved for this population, in this setting

 

 

Table 7a: Organism-Directed Antibiotic Therapy

Organism

Recommended Antibiotic(s)

 

Alternative(s)

Comment(s)

S. pneumoniae

 

 

 

Penicillin (PCN) MIC<0.1 mg/L

PCN G or ampicillin

cefotaxime, ceftriaxone, chloramphenicol

 

 

 

 

 

PCN MIC 0.1-1.0 mg/L

cefotaxime or ceftriaxone

cefepime, meropenem

 

 

 

 

 

PCN MIC ≥2.0mg/L

vancomycin plus ceftriaxone/cefotaxime (plus rifampin if dexamethasone is used)

moxifloxacin, levofloxacin

Regimen for meningitis; clinical data unavailable for fluoroquinolones

 

Cefotaxime or ceftriaxone MIC ≥1.0mg/L

vancomycin plus ceftriaxone/cefotaxime ± rifampin

moxifloxacin, levofloxacin

Regimen for meningitis; clinical data unavailable for fluoroquinolones

 

 

 

 

H. influenzae (type b and others)

 

 

 

Beta-lactamase negative

ampicillin

cefotaxime, ceftriaxone, cefepime, chloramphenicol, moxifloxacin, levofloxacin, ciprofloxacin

 

 

Beta-lactamase positive

ceftriaxone, cefotaxime

cefepime, chloramphenicol, moxifloxacin, levofloxacin, ciprofloxacin

 

 

 

 

 

N. meningitidis

 

 

 

PCN MIC <0.1 mg/L

PCN G or ampicillin

ceftriaxone, cefotaxime, chloramphenicol

 

 

 

 

 

PCN MIC 0.1-1.0 mg/L

ceftriaxone, cefotaxime

Chloramphenicol, moxifloxacin, levofloxacin, ciprofloxacin, meropenem

 

 

 

 

 

Salmonella species

Ceftriaxone, cefotaxime, ciprofloxacin, levofloxacin

ampicillin, imipenem, meropenem, ertapenem

Use ampicillin only once sensitivities are known.  Meropenem is preferred agent for CNS infections

 

 

 

 

Capnocytophaga canimorsus

Amoxicillin-clavulinic acid, ampicillin-sulbactam

Imipenem, ertapenem, meropenem, clindamycin, rifampin, doxycycline, erythromycin, vancomycin, cefotaxime, ceftriaxone, ciprofloxacin, levofloxacin

Resistant to aztreonam.

 

 

 

 

 Bordetella holmesii

imipenem, carbapenem, ciprofloxacin, levofloxacin

 

Beta-lactams are not advised.

 

 

 

 

Babesia microti

Atovaquone plus azithromycin

Clindamycin plus oral quinine

For dosages, see table 7b.

 

 

 

 

Plasmodia spp.

www.cdc.gov/ncdidod/dpd/parasites/malaria/default/htm

 

 

 

Severe, non-pregnant

quinidine gluconate plus doxycycline or clindamycin

and primaquine (for non-falciparum spp.)

 

For dosages, see table 7b.

Pregnant, not severe

quinine sulfate plus clindamycin

Quinine sulfate plus doxycycline (if preferred regimen failing)

primaquine, if necessary, after delivery to treat non-falciparum; avoid mefloquine; for dosages, see table 7b.

 

Table 7b: Usual Dosages of Antibiotics

Antibiotic (trade name)

 

Adult dosage

Pediatric dosage

Renal adjustment

Comments

Amoxicillin-clavulanate (Augmentin)

500/125-875/125 mg PO twice daily with food OR

1000/62.5 2 tabs PO twice daily with food [Augmentin XR]

25-50 mg/kg PO divided every 8 hrs OR

600/42.9/5 ml

90 mg/kg divided PO every 12 hrs with food [ES-600]

Reduce frequency to once or twice per day.  Augmentin XR contraindicated with CrCl <30.

 

 

Ampicillin

2 gr IV every 4-6 hrs

50 mg/kg IV every 6 hours

Reduce to every 8 to 24 hrs

 

 

Ampicillin-sulbactam (Unasyn)

3 gr IV every 6 hrs

100-300 mg/kg every 6 hrs

Reduce to every 8-24 hrs

 

 

Atovaquone (Mepron)

750 mg (5 ml) PO twice daily for 7-10 days

 

 

Tx for Babesiosis

 

 

Azithromycin (Zithromax)

600 mg PO daily for 7-10 days

 

 

Tx for Babesiosis

 

Cefepime (Maxipime)

2 gr IV every 8-12 hrs

150 mg/kg IV div every 8 hrs

Reduce frequency to 12-24 hrs

 

 

Chloramphenicol

50-100 mg/kg IV div every 6 hrs

12.5-50 mg/kg IV every 6 hrs (2-4 gr max)

Not applicable

 

 

 

 

Clindamycin (Cleocin)

1.2 gr IV every 12 hr hrs for 7-10 days

25 mg/kg IV every 12 hrs for 7-10 days

Not applicable

Tx for Babesiosis

 

Doxycycline (Vibramycin)

100 mg PO/IV every 12 hrs

2-4 mg/kg PO/IV div every 12 hrs (max 200 mg per day); avoid in children less than 8 y/o

Not applicable

 

 

 

 

 

 

 

Ertapenem (Invanz)

1 gr IV daily

30 mg/kg IV div every 12 hrs

Decrease amount by 50%

 

 

 

 

Erythromycin

250-500 mg PO every 6 hrs

10 mg/kg IV/PO every 6 hrs

Not applicable

 

 

 

Penicillin

4 million units IV every 4 hrs

250,000-400,000 units/kg IV div every 4-6 hrs

Adults: decrease frequency to 8-12 hrs

Peds: decrease dose 25-50%

 

 

 

 

 

 

 

 

Primaquine

30 mg PO daily for 14 days

0.6 mg/kg daily for 14 days

Not applicable

Check G6PD level

 

Quinine (oral)

650 mg PO every 6-8 hrs for 7-10 days

25 mg/kg PO div every 6-8 hrs for 7-10 days

 

Tx for Babesiosis and Plasmodia spp.

 

Quinidine gluconate

10 mg/kg (salt) IV in NS over 1 hr, then 0.02 mg/kg IV continuous

Same as adult

Change to quinine sulfate when  <1% parasite load

 

 

 

 

 

Quinine sulfate

648 mg po every 8 hrs for 3-7 days

30 mg/kg PO div every 8 hrs for 3-7 days

Load 648 mg, then 324 mg every 12 hrs

 

 

Table 8:  Recommended Vaccines

Vaccine

Adult

Pediatric

Booster

Comment

 

 

 

 

 

S. pneumoniae

 

 

 

 

Pneumococcal conjugate vaccine (PCV-7)

(Prevnar)

See PPV

Age ≤23 months: 4 doses at 2, 4, 6 and 12-15 months (0.5 ml IM thigh or deltoid)

PPV-23 at age ≥ 2 y/o

Give ≥2 mos after last PCV; 0.5 ml IM/SC; repeat 3-5 yrs later

 

PCV not for use in children >59 months old

Pneumococcal polysaccharide vaccine (PPV-23)

(Pneumovax)

0.5 ml IM/SC

See PCV

Repeat ≥5 yrs

If age ≥65 and ≥5 yrs since last dose

Only two doses in lifetime are recommended

 

 

 

 

 

Haemophilus influenzae type b (Hib)

 

 

 

Hib/DTaP (TriHIBit) and Hib/HepB (COMVAX) are also available

 

PRP-OMP (PedvaxHIB)

0.5 ml IM

Two doses two months apart beginning at 2 months old (0.5 ml IM)

Age 12-15 months (may use TriHIBit also) (0.5 ml IM)

No clinical data for adults, but appears well tolerated; only one dose is recommended

 

HbOC (HibTITER), PRP-T (ActHIB),

0.5 ml IM

6 weeks to 71 months: Three doses two months apart beginning at 2 months old (0.5 ml IM)

 

Age 12-15 months (may use TriHIBit also) (0.5 ml IM)

No clinical data for adults, but appears well tolerated; only one dose is recommended

 

 

 

 

 

Neisseria meningitides

 

 

 

CDC continues to recommend; please see comments in text.

 

Quadravalent polysaccharide vaccine (MPS4) (Meromune)

0.5 ml SC

Age ≥ 2 years;

0.5 ml SC

Provides protection for 3-5 yrs ; revaccinate for high risk situations

 

Covers A,C, Y and W-135

Quadrivalent conjugate vaccine (MCV4) (Menactra)

 

Ages 11-55 ;

0.5 ml IM deltoid

Age ≥ 1 ;

0.5 ml IM deltoid

Unknown

Covers A,C, Y and W-135

Monovalent MC type B

 

 

 

Not available in United States

 

 

 

 

 

Influenza virus

 

 

 

 

 

Injectable (Fluvirin, Fluzone,

Fluarix)

0.5 ml IM deltoid

Ages 6 months-8 yrs: 2 injections at least 4 weeks apart for first time recipients; ages 6-35 months: 0.25 ml IM, ant-lat thigh

Ages 3-8 yrs: 0.5 ml IM ant-lat thigh or deltoid (older children)

Yearly

Fluvirin approved for ≥5 yrs; Fluarix approved for ≥18; optimally administer October-

November

Intranasal (LAIV)

(Flumist)

Age ≥ 12 years but  ≤ 49 years: 0.5 ml intranasal

Ages 5-8: two doses 0.5 ml intranasal 6-10 weeks apart; if first dose was injectable, wait 4 weeks.

Ages ≥9 years: 0.5 ml intranasal

Yearly

Avoid in pts severely immuno-

compromised or in those who will be exposed to severely immuno-

compromised

 

Table 9: Prevention

 

Immunization:  S. pneumoniae, Influenza virus, Hib; possibly N. meningitidis

Tick, mosquito and animal bite prevention

Oral antibiotic prophylaxis

Availability of medical and contact information, i.e, medical bracelet

Early intervention with appropriate support and antibiotics

 

Figure 1

 

Figure 2

 

Figure 3

 

Figure 4

 

Figure 5