fungal sinusisit with macrophage activation syndrome
TRANSCRIPT
Fungal sinusisit with Macrophage Activation Syndrome
Dr madan guptaENT & HNSAll India institute of medical sciences
26/m c/o-Swelling over face and nose -40
days fever-40 days
History
2nd june- ESS (pan sinusitis)[intra op finding-septal perforation and sinus was filled with purulent discharge]
After 10th day of surgery-swelling over nose and face
Started on conservative management ….but no response
Referred to AIIMS
Examination – Temp-febrile pallor- mild Icterus- no Clubbing-noHepatosplenomegaly+L/E-Diffuse midline swelling over the nose,
extending inf up to lower lip and sup up to lower lid(B/L)
Nose-septal perforation and b/l nasal cavity filled with crust
Neck-wnl Oc-wnl Ear-wnl Larynx-wnl
D/D
wegener’s granulomatosis NK/T cell lymphoma fungal infection
Investigation
ANCA-negative URINE R/M-wnl Nasal BX- necrotic mass with aspergillus
fungus Glactomannan test-positive TB pcr-negative IRCH Haemogram-RBC-normocyte ,
normochromic, WBC-N85 L11 M4 , Reticulocyte -1% PLT-1.5 lakh No abnormal cell or parasite seen
HIV-negative Viral marker-negative VDRL & TPHA-negative USG abd-enlarged spleen (14.5 cm) CECT PNS-B/l maxillary,ethmoid &
sphenoid sinusitis. CECT chest (RC discussion)-
multifocal hemorrhage??vasculitis or fungal infection.
BM aspirate-cellular reactive,all hemopoetic element,increase in number of histiocytes showing hemophagocytosis
BM Bx-prominenence of histiocytes,no grnuloma or lymphoma
28/7 6/8 12/8 16/8 17/8 18/8 19/8 20/8
HB 9.5 9.4 8.6 7.4 7.8 6.7 6.3 7.6
TLC 4700 4600 5200 2800 2500 2000 1700 2400
N 69 76 80 84
L 19 17 11 13
M 11 6 7 15
PLT 117000 103000 158000 105000 91000 71000 100000 43000
ESR 110 67 57 36
urea 28 23 28 29 32 38 40 44
creat 0.8 0.6 0.5 0.5 0.6 0.9 0.5 0.8
biliru 0.6 0.5 0.4 0.6 0.7 0.1 1.3
ALP 177 189 199 802 1773
alb 3.5 2.8 2.8 2.3 1.9
globu 3 2.6 2.9 2.1 2.9
POSITIVE FINDING
Persisting fever Splenomegaly Pancytopenia Hemophagocytes in BM Triglyceride-173mg/dl Aspergillus fungal in culture Glactomannan -positive
Histiocytic Society Protocol Criteria
1. Fever(>7days)
2. Splenomegaly
3. Cytopenias(>2 lineages)
-Anemia(hb<9.0 g/dl)
-Neutropenia(<1000)
-Thrombocytopenia(<1lk cells)
4. Hypertriglyceridemia & Hypofibrinigenemia
5.Haemophagocytosis(bone, spleen,bone marrow)
6. Natural killer cell activity(low/absent)
7.Hyperferritinemia(>500 mcg/l)
8. Increased soluble CD 25(>2400 u/ml)
diagnosis
Fungal sinusisit with Macrophage Activation Syndrome
IV dexa IVAmphoVoriconazoleAntibiotic
Initial improvement showed but patient expired due to sepsis with septic shock.
discussion
Macrophage Activation Syndrome or Hemophagocytic Syndrome
haemophagocytosis- pathologic finding of activated macrophages engulfing erythrocytes, leukocytes,platelets, and their precursor cells.
PRIMARY(Familial)
SECONDARY(Acquired)
INFECTIONIMMUNODE-FICIENCY
AUTOIMMUNEMETABOLIC
DS.MALIGNANC
Y
ETIOLOGY
PATHOPHYSIOLOGY
TRIGGERING OF THE CYTOKINE CASCADE
FREE OXYGEN RADICAL RELEASE
ACTIVATED MACROPHAGES PHAGOCYTOSE RBCS,WBCS,PLATELETS
INAPPROPRIATE ACTIVATION & UNCONTROLLED PROLIFERATION OF THE MACROPHAGES
TRIGGERING FACTOR (MC INFECTION)
CLINICAL FEATURES
Onset- abrupt Many present with fever of unknown origin. Systemic manifestations-pallor,fever,rash,
lymphadenopathy,hepatosplenomegaly,neurological manifestations.
It takes a fulminant course and has a fatal outcome.
Work-up
Bacterial: Bl Cx, U Cx, Viral pathogens: EBV, CMV, parvo, HIV Fungal Cx and serology Eval for lymphoproliferative DO – BM bx Recent Travel or animal exposure – eval for
Leishmaniasis, brucellosis, rickettsioses, malaria
HIV +: serum crypto ag,
Treatment
Steroids + Etoposide + Cyclosporine A Other considerations
ATG IVIG
Bone Marrow Transplant Familial Disease Non-familial: only if fail immuno-/chemo-
therapy
Prognosis
Mortality 22-59% Prognostic Factors predicting death
>30 yr Underlying disease process Hb <10 Platelet <100 k Ferritin > 500 ug/l Bili or alk phos elevation