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