Kawasaki with hlh,an unusual case of fever
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Transcript of Kawasaki with hlh,an unusual case of fever
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AN UNUSUAL CASE OF PROLONGED FEVER
SPEAKER - Dr. GNANDAS BARMAN
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Pritwish Mondal1 year old Male/Hindu child fromBongaonAdmitted on 07/09/2014
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Chief Complaints: •Fever and excessive irritability for -10 days
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H/O Present illnessPatient developed high grade continous fever
1o days prior to admission. Associated redness of both eyes. Fever was not associated with rigor, cough,
rash, dysuria, altered sensorium, convulsion.The child was extremely irritable since the
onset of fever.
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On Examination:Alert and irritable.Vitals- PR-140/min, BP-80/44mm of Hg, RR-36/min, Temp-104.2⁰F, CRT-2 sec SpO2-97% in room air.AF- closed Bilateral bulbar conjunctival congestion.Pallor present
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On Examination:Multiple right posterior cervical
lymphadenopathy- 1.5cm, firm, discrete, mobile ,
nontender.No rash /desquamation.
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On Examination:-Anthropometry: - Wt- 10.5 Kg (between 50th - 85th
percentile) Length - 78cm (50th - 85th percentile)
Head circumference-45 cm (15th - 50th percentile)
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G.I. SYSTEM- UPPER G.I.- normal
Liver -4 cms along Rt MCL , liver span-10 cms, firm, sharp margin, non tender,
left lobe not palpable. Spleen-3 cms Other systems- WNL
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Differential DiagnosisVIRAL FEVER.
HEMATOLOGICAL MALIGNANCIES. KAWASAKI DISEASE
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INVESTIGATIONS (7/9/14):CBC- Hb: 6 gm% TLC: 20,000/ cu mm,
(N60/L36/M2/E2) Platelet: 50,000/cumm ESR- 25 mm, no abnormal cells in peripheral smear.
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LFT: TSB-0.6, ALT/AST-80/68, Alb-2.6,Glb-2.8Urea/Creatinine-22/0.8 Serum electrolytes-Na/K- 142/4.7Urine RE/ME: 5-6 pus cells.
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MP slide, MPDA- Negative
Dengue IgM-NR
Widal- Negative.
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Urine and blood c/s sent.
Mantoux test done.
Chest X ray- WNL
USG W/A-Hepatosplenomegaly
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Treatment:-IV FluidsInj CeftriaxoneSyr ParacetamolPRBC transfusion
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On 10/9/14
Child toxic and having high grade fever.
INVESTIGATIONS -Blood and urine cultures- sterile, Mantoux- negative
CSF -04 lymphocytes/cu mm, sugar-80 mg/dl, protein-24mg/dl, ADA- 0.9.
CBC –Hb -10.5 gm% TLC- 22,000 (N62 L32 E2 M4)
Platelets-48,000/cmm,
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Bone marrow aspiration study done.
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11/9/14ECHOCARDIOGRAPHY- RIGHT CORONARY ARTERY
ANEURYSM6mmLVEF-52%; mild LV systolic dysfunction.No valvular regurgitation or pericardial effusion. - Highly suggestive of Kawasaki
disease.
PBS platelets-45,000
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ON 12/09/14Serum Ferritin-1886 ng/ml(>500)Triglyceride-442mg/dl(>265 )Fibrinogen-119.6mg/dl(<150)
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BONE MARROW STUDY-Bone marrow examination showing
Hemophagocytosis
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PROPOSED HLH DIAGNOSTIC CRITERIA,2009:
[1] Molecular diagnosis of hemophagocytic lymphohistiocytosis(HLH) or X-linked lymphoproliferative syndrome (XLP).
[2] Or at least 3 of 4:a. Feverb. Splenomegalyc. Cytopenia (minimum 2 cell lines reduced)d. Hepatitis
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PROPOSED HLH DIAGNOSTIC CRITERIA,2009:
[3]. And at least 1 of 4:a. Hemophagocytosisb. ↑ Ferritinc. ↑ sIL2Rα (CD25)d. Absent or very decreased NK function[4]. Other results supportive of HLH diagnosis:a. Hypertriglyceridemiab. Hypofibrinogenemiac. Hyponatremia
Filipovich A et al[ASH Education Book Jan 1,2009 vol.2009 no. 1 127-131]
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DIAGNOSIS ATYPICAL KAWASAKI DISEASE WITH
SECONDARY HEMOPHAGOCYTIC
LYMPHOHISTIOCYTOSIS[HLH]
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TREATMENTIV IMMUNOGLOBULIN- 2gms/kg
transfused over 24 hours on 13/9/14.
Platelet count-80,000 on 14/9/14CBC on 15/9/14 : Hb-11, Tc-
12,000,N52L42B5E1 platelets-1.5 lakh
Aspirin 80 mg/kg started from 15/9/14.
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Patient became afebrile 36 hrs after IVIG transfusion.
Hepatosplenomegaly started to regress within 3 days of IVIG transfusion
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Follow up
@6 wks
Echo-normal coronaries Aspirin stopped
Aspirin continued for 6 wks @5mg/kg/day
Two weeks later
Echo –no abnormality Aspirin dose reduced to 5 mg/kg/day
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DISCUSSION 1.9% of children with acute kawasaki
disease are reported to develop secondary HLH.†
It result from cytotoxic dysfunction leading to persistent expansion of T cells and Macrophages , escalating production of proinflammatory cytokines.
†Latino et al[ J Pediatr Hemato Oncol 2000
oct;32(7):527-31]
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Present with prolonged and persisting fever beyond initial IVIG treatment ; KD complicated with secondary HLH is difficult to distinguish from refractory KD/ recurrent KD.
Onset of secondary HLH or MAS with mean of 13.3 days [range 3-22 days];
However recurrent KD typically occurred much later at a mean of 17.9 months[range 1-60 months]‡
Treatment includes Pulse Methyl Prednisolone, Anakinra, Etoposide.
‡ Kang et al[Blood Res. 2013 Dec; 48(4):
254–257]
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1.Latino et al[ J Pediatr Hemato Oncol 2000 oct;32(7):527-31]
2. Wang et al[Semin Arthritis Rheumatism 2014 aug;44(4):283-304]
CASE SERIES
PATIENTS OF KD COMPLICATED WITH HLH
RESPONSIVE TO IVIG
ADDITIONAL IMMUNOMODULATOR REQUIRED
1. 12 1 11
2. 8 1 7
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THANK YOU