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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
Objective
  • To present a case of a Hemophagocytic Lymphohistiocytosis (HLH)
general data
General Data
  • BM
  • 6 year old
  • Male
  • from Bacoor, Cavite
slide4
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

slide5
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

past medical history
Past Medical History
  • no previous hospitalization
  • (+) asthma
  • no known allergies
family history
Family History
  • (-) asthma, allergies
  • (-) cancer
  • (-) DM, HPN
immunization history
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

physical exam
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
slide10
CBC done:
  • Dengue blot negative
  • Admitted as a case of Systemic Viral Illness r/o Dengue fever
  • IV hydration, Paracetamol
course in the wards
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
course in the wards2
Course in the Wards
  • Cardiac: ECG: Normal sinus rhythm, normal axis
course in the wards3
Course in the Wards
  • Respiratory: CHEST XRAY Bilateral Interstitial Pneumonitis)

Ceftriaxone 1.5g/IV OD

course in the wards4
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
course in the wards5
Course in the Wards

Sodium correction

Albumin transfusion

course in the wards6
Course in the Wards
  • REFERRALS
        • IDS: Cannot totally rule out Dengue fever; HLH
        • GI : Systemic Viral Illness, Postinfectious hepatitis
        • HEMATOLOGY: t/c HLH
transfer to picu
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

transfer to picu1
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

diagnostic guidelines for hlh
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.

at the picu
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
at the picu1
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)
at the picu2
At the PICU
  • BMA: Hemagophagocyte - 1
  • for flow cytometry
hemophagocytic lymphohistiocytosis
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

hemophagocytic lymphohistiocytosis1
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.

slide27
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).

slide28
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

slide29
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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