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Objective

Objective. To present a case of a Hemophagocytic Lymphohistiocytosis (HLH). General Data. BM 6 year old Male from Bacoor, Cavite. Chief complaint. FEVER. History of Present Illness. (+) fever (Tmax: 40degrees C) (+) occasional cough (+) headache and joint pains

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Objective

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  1. Objective • To present a case of a Hemophagocytic Lymphohistiocytosis (HLH)

  2. General Data • BM • 6 year old • Male • from Bacoor, Cavite

  3. Chief complaint FEVER

  4. History of Present Illness (+) fever (Tmax: 40degrees C) (+) occasional cough (+) headache and joint pains Paracetamol for the fever 5 days PTA (+) persistence of fever Consult done at St. Dominic Hospital CBC: Hgb 122/ Hct 37/ WBC 7.27/ neutro 63/ lymph 24/ platelet 289 UA: normal Dengue NS1 negative Impression: Upper respiratory tract infection Clarithromycin 3 days PTA

  5. History of Present Illness (+) persistence of fever (+) abdominal pain, nausea, myalgia Follow-up consult St. Dominic hospital rpt CBC: Hgb 110/ Hct 35/ wbc 1.7/ neutro 36/ lymph 58/ platelet 95 Typhidot: negative Advised admission, but opted transfer to our institution On the day of admission ADMISSION

  6. Past Medical History • no previous hospitalization • (+) asthma • no known allergies

  7. Family History • (-) asthma, allergies • (-) cancer • (-) DM, HPN

  8. Immunization History BCG 1 dose Hepatitis B 3 doses DPT 3 doses OPV 3 doses Pneumococcal none Rotavirus none Hepatitis A 1 dose Typhoid none

  9. Physical exam • Awake, weak-looking , not in cardiorespiratory distress • BP: 90/60mmHg CR: 98bpm RR: 24 cpm T: 39.6 C • Weight: 19.8 kg • Height 131 cm • pink palpebral conjunctivae, anicteric sclerae • moist oral mucosa, no tonsillopharyngeal congestion • good air entry, clear breath sounds • regular cardiac rhythm, grade 2/6 systolic murmur on left parasternal border • soft abdomen, nontender • full and equal pulses, CRT <2sec

  10. CBC done: • Dengue blot negative • Admitted as a case of Systemic Viral Illness r/o Dengue fever • IV hydration, Paracetamol

  11. Course in the Wards • Problems: • (+) persistence of fever • occasional cough • increased effort in breathing • with episodes of abdominal pain • cardiac findings: Grade 2/6 systolic murmur on left parasternal border • respiratory: RR 30-60s, harsh breath sounds, no rales, no wheezes • abdominal findings: soft abdomen, liver edge palpable 3-4 cm below the subcostal margin

  12. Course in the Wards • Infectious

  13. Course in the Wards • Cardiac: ECG: Normal sinus rhythm, normal axis

  14. Course in the Wards • Respiratory: CHEST XRAY Bilateral Interstitial Pneumonitis) Ceftriaxone 1.5g/IV OD

  15. Course in the Wards • Abdomen: PLAIN ABDOMINAL XRAY: No localizing signs in the abdominal tissues • ABDOMINAL ULTRASOUND prominent sized liver, mild to moderate ascites and pleural effusion

  16. Course in the Wards Sodium correction Albumin transfusion

  17. Course in the Wards • REFERRALS • IDS: Cannot totally rule out Dengue fever; HLH • GI : Systemic Viral Illness, Postinfectious hepatitis • HEMATOLOGY: t/c HLH

  18. Transfer to PICU Meropenem 750 mg/IV every 8h (113.6mkd) • prolonged fever • tachypnea RR 50s and labored breathing • tachycardia HR 110-120s bpm • still with episodes of abdominal pain O2 support 2lpm NC 2d-echo: pericardial effusion, mild MR, mild TR Carditis with diastolic dysfunction t/c SEPSIS rule out HLH

  19. Transfer to PICU pRBC transfusion • anemia, leukopenia, thrombocytopenia • electrolyte imbalance (hypokalemia, hypocalcemia) • fluid spacing (pleural effusion, ascites) • deranged liver function tests K correction Calcium gluconate Furosemide

  20. Other laboratory results

  21. Diagnostic Guidelines for HLH Text Treatment Protocol of the 2nd International HLH Study, 2004.)From Verbsky JW, Grossman WJ: Hemophagocytic lymphohistiocytosis: diagnosis, pathophysiology, treatment, and future perspectives, Ann Med 38:20–31, 2006, p 21, Table 1 Retrieved from Nelsons Pediatrics 19th ed.

  22. At the PICU • CT Abdomen with Contrast: • Prominent sized liver and spleen • Liver 13.5 cm right midclavicular line • Spleen 8.2 x 8.8 x 4.3 cm • Ascites • Bilateral pleural effusion, right more than left

  23. At the PICU • Triglycerides 460.20 mg/dl (nv 0-150) • Fibrinogen 136.2 mg/dl (nv 180-350) • Ferritin >40,000 ng/ml (nv 8.80 - 184.7)

  24. At the PICU • BMA: Hemagophagocyte - 1 • for flow cytometry

  25. Hemophagocytic Lymphohistiocytosis • also called “hemophagocytic syndrome (HPS) • a nonmalignant proliferative disorder that affects the antigen-processing macrophages and that results in uncontrolled hemophagocytosis and upregulation of inflammatory cytokines impaired natural killer (NK) cell function and other defects  Palazzi D L et al. Hemophagocytic Syndrome in Children: An Important Diagnostic Consideration in Fever of Unknown Origin. Clin Infect Dis. 2003;36:306-312

  26. Hemophagocytic Lymphohistiocytosis • A potentially fatal disorder of children and adults due to cytokine dysfunction, resulting in uncontrolled accumulation of activated T-lymphocytes and activated histiocytes (macrophages) in many organs. • HLH may be familial, associated with a number of different infections, autoimmune disorders, or coincident with a number of malignancies. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis.AU Henter JI, Horne A, AricóM, Egeler RM, Filipovich AH, Imashuku S, Ladisch S, McClain K, Webb D, Winiarski J, Janka G SO. Pediatr Blood Cancer. 2007;48(2):124.

  27. Primary HLH • Familial hemophagocytic lymphohistiocytosis (FHLH) (familial or sporadic): • an autosomal recessive disease that affects immune regulation • Nonfamilial HLH: • develop from marked immunological activation during viral, bacterial, and parasitic infections • may also be associated with malignancies, prolonged administration of lipids, rheumatoid arthritis (macrophage activation syndrome), immune deficiencies associated with cytotoxic T- and/or Nkcell dysfunction such as DiGeorge syndrome (del 22q11.2), Chédiak–Higashi syndrome, Griscelli syndrome,* X-linked lymphoproliferative disease (XLP), and lysinuric protein intolerance (LPI). Manual of Pediatric Hematology and Oncology 4th ed . P. Lanzkowsky (Elsevier, 2005).

  28. Secondary HLH • A reactive disorder causing strong immunologic activation often resulting from severe bacterial or parasitic infection • Infection-associated HPS IAHS] • Viral infection (VAHS) • Malignancy (MAHS) • Use of drugs (phenytoin) • Prolonged administration of parenteral nutrition involving soluble lipids Palazzi D L et al. Hemophagocytic Syndrome in Children: An Important Diagnostic Consideration in Fever of Unknown Origin. Clin Infect Dis. 2003;36:306-312

  29. Infection-Associated Hemophagocytic Lymphohistiocytosis • NK-cell activity in IAHLH patients is reconstituted as soon as the infection is cleared • decreased or absent NK cells are found more often in FHLH • Viruses: • Epstein–Barr virus, human herpes virus 6 (HHV-6), cytomegalovirus (CMV) (most common of the viruses), adenovirus, parvovirus, varicella zoster, herpes simplex virus (HSV), Q-fever virus, and measles • Treatment • EBV–related IAHLH: etoposide and immunoglobulin treatment • Other infections: antibiotics for bacterial infections, antiviral drugs for viruses, in addition to corticosteroids and/or etoposide. • Patients with persistent HLH may require FHLH treatment and HSCT. • Patients with resolved disease may discontinue therapy at 8 weeks. If the syndrome recurs therapy should be restarted and HSCT should be employed.

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