International Journal of Clinical Pediatrics, ISSN 1927-1255 print, 1927-1263 online, Open Access
Article copyright, the authors; Journal compilation copyright, Int J Clin Pediatr and Elmer Press Inc
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Case Report

Volume 9, Number 1, March 2020, pages 24-29

Bone Marrow Necrosis in a Child With Sepsis-Induced Macrophage Activation Syndrome

Figure

Figure 1.
Figure 1. (a) Bone marrow aspirate smears were particulate and appeared normocellular (May-Grunwald Giemsa, × 40). (b) The background showed mostly necrotic cells with ill-defined cellular boundaries admixed with fat and very few viable cells (May-Grunwald Giemsa, × 200). (c) High power examination revealed several histiocytes with ingested erythrocytes, lymphocytes and cell debris (May-Grunwald Giemsa, × 400). Ziehl-Neelsen (ZN) stain for acid-fast bacilli (d) and periodic acid-Schiff stain for fungi (e) were negative, but highlighted the phagocytic histiocytes (× 400, ZN stain counterstained with methylene blue, PAS stain counterstained with hematoxylin). (f) Chest radiograph showed bilateral pulmonary edema secondary to transfusion-related lung injury. (g) Graphs showing trends of the blood counts during hospital stay.

Tables

Table 1. Laboratory Parameters During the Hospital Course
 
InvestigationsAt admissionDay 3Day 7Day 10
PT: prothrombin time; INR: international normalized ratio; aPTT: activated partial thromboplastin time; CRP: C-reactive protein.
Complete blood counts
  Hemoglobin (g/L)741089561
  Total leukocyte count (× 109/L)1,7002,1102,49028,840
  Neutrophils (%)-7.62.984.7
  Lymphocytes (%)-87.293.411.4
  Monocytes (%)-5.2-3.7
  Eosinophils (%)--0.8-
  Basophils (%)----
  Platelets (× 109/L)38,0004,00028,00053,000
Renal function test
  Urea (mg/dL)6510896107
  Creatinine (mg/dL)0.70.730.50.54
Coagulogram
  PT (seconds)211714.8
  PTI (%)626676
  INR2.531.531.33
  aPTT (s)37
  D-dimer (ng/mL)50904741
MAS parameters
  Ferritin (ng/mL)2,6233,245
  Fibrinogen (g/L)5.723.41
  Triglycerides (mg/dL)266156
Inflammatory parameters
  CRP (mg/L)195.7194173
  Procalcitonin (ng/mL)49.4832.49
Microbiological investigations
  Blood cultureDay 1: methicillin-resistant Staphylococcus aureus
Day 6: Escherichia coli
Day 8: Candida tropicalis
  Pus cultureDay 2: methicillin-resistant Staphylococcus aureus

 

Table 2. Clinical Details of Children With Infection-Induced Bone Marrow Necrosis Reported in Literature
 
Kinney et al [5]Terheggen et al [6]Garewal et al [7]Garewal et al [7]Index case
HSM: hepatosplenomegaly; MRSA: methicillin-resistant Staphylococcus aureus; IVIG: intravenous immunoglobulin; TRALI: transfusion-related acute lung injury.
Year of reporting19771979199119912020
Age (years)1582.53.510
SexFemaleMaleMaleMaleFemale
Clinical presentationMigratory arthralgia, epistaxis, weight loss, polyarthritis, fever, hepatomegalyFever, furuncle, joint and limb pain, HSMPallor, mucocutaneous gangrene, popliteal ulcer, HSMFever, epistaxis, oral ulcer, pallor, pneumonia, HSMFever, erythematous rash, epistaxis, pneumonia, chest wall abscess, hepatomegaly, shock
Hemoglobin (g/dL)5.6124.35.27.4
Total leukocyte count (× 109/L)7.052.52.01.61.7
Absolute neutrophil count (× 109/L)3.9-0.80.20.6
Platelet count (× 109/L)8678101031
Blood cultureStaphylococcus aureusGroup A StreptococcusPseudomonas aeruginosaSalmonella typhimuriumMRSA
Features suggestive of macrophage activation syndromeFever, hepatomegaly, anemia, thrombocytopeniaFever, HSM, thrombocytopenia, leukopeniaHSM, pancytopeniaFever, HSM, pancytopeniaFever, hepatomegaly, pancytopenia, hyperferritinemia, hypertriglyceridemia
TreatmentAntibiotics and prednisoloneAntibioticsAntibioticsAntibioticsAntibiotics, IVIG, methylprednisolone pulse
OutcomeImproved, developed breast lymphosarcoma after 11 months and ultimately died of it.Discharged on day 13, no long-term follow-upDischarged on day 12, no long-term follow-up.Developed septic arthritis and skin abscesses, and died.Initially shown improvement, died on day 12 due to TRALI.