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Fungal sinusisit with Macrophage Activation Syndrome

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madan gupta
madan gupta

A 26-year-old male presented with swelling of the face and nose as well as fever for 40 days. He had undergone endoscopic sinus surgery previously for pansinusitis but symptoms persisted. Examination found diffuse swelling of the nose and face. Investigation revealed fungal sinusitis with Aspergillus and macrophage activation syndrome (MAS) based on persistent fever, splenomegaly, pancytopenia, hemophagocytosis in bone marrow, and positive glucatoman test. The patient was treated with antifungals and steroids but expired due to sepsis and septic shock. MAS is a severe hyperinflammatory condition caused by inappropriate activation of macrophages leading to hemophagocytosis and multi-organ

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Fungal sinusisit with Macrophage Activation Syndrome Dr madan gupta ENT & HNS All 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-no Hepatosplenomegaly+ 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 wegeners 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(bon e, spleen,bone marrow) 6. Natural killer cell activity(low/absent) 7.Hyperferritinemia(>50 0 mcg/l) 8. Increased soluble CD 25(>2400 u/ml)
diagnosis Fungal sinusisit with Macrophage Activation Syndrome IV dexa IVAmpho Voriconazole Antibiotic 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) INFECTION IMMUNODE -FICIENCY AUTOIMMUNE METABOLIC DS. MALIGNANCY ETIOLOGY
PATHOPHYSIOLOGY TRIGGERING FACTOR (MC INFECTION) INAPPROPRIATE ACTIVATION & UNCONTROLLED PROLIFERATION OF THE MACROPHAGES TRIGGERING OF THE CYTOKINE CASCADE FREE OXYGEN RADICAL RELEASE ACTIVATED MACROPHAGES PHAGOCYTOSE RBCS,WBCS,PLATELETS
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

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Fungal sinusisit with Macrophage Activation Syndrome

  • 1. Fungal sinusisit with Macrophage Activation Syndrome Dr madan gupta ENT & HNS All India institute of medical sciences
  • 2. 26/m c/o- Swelling over face and nose -40 days fever-40 days
  • 3. 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
  • 4. Referred to AIIMS Examination Temp-febrile pallor- mild Icterus- no Clubbing-no Hepatosplenomegaly+ L/E-Diffuse midline swelling over the nose, extending inf up to lower lip and sup up to lower lid(B/L)
  • 5. Nose-septal perforation and b/l nasal cavity filled with crust Neck-wnl Oc-wnl Ear-wnl Larynx-wnl
  • 6. D/D wegeners granulomatosis NK/T cell lymphoma fungal infection
  • 7. 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
  • 8. 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.
  • 9. BM aspirate-cellular reactive,all hemopoetic element,increase in number of histiocytes showing hemophagocytosis BM Bx-prominenence of histiocytes,no grnuloma or lymphoma
  • 10. 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
  • 11. POSITIVE FINDING Persisting fever Splenomegaly Pancytopenia Hemophagocytes in BM Triglyceride-173mg/dl Aspergillus fungal in culture Glactomannan -positive
  • 12. 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(bon e, spleen,bone marrow) 6. Natural killer cell activity(low/absent) 7.Hyperferritinemia(>50 0 mcg/l) 8. Increased soluble CD 25(>2400 u/ml)
  • 13. diagnosis Fungal sinusisit with Macrophage Activation Syndrome IV dexa IVAmpho Voriconazole Antibiotic Initial improvement showed but patient expired due to sepsis with septic shock.
  • 14. discussion Macrophage Activation Syndrome or Hemophagocytic Syndrome haemophagocytosis- pathologic finding of activated macrophages engulfing erythrocytes, leukocytes,platelets, and their precursor cells.
  • 15. PRIMARY (Familial) SECONDARY (Acquired) INFECTION IMMUNODE -FICIENCY AUTOIMMUNE METABOLIC DS. MALIGNANCY ETIOLOGY
  • 16. PATHOPHYSIOLOGY TRIGGERING FACTOR (MC INFECTION) INAPPROPRIATE ACTIVATION & UNCONTROLLED PROLIFERATION OF THE MACROPHAGES TRIGGERING OF THE CYTOKINE CASCADE FREE OXYGEN RADICAL RELEASE ACTIVATED MACROPHAGES PHAGOCYTOSE RBCS,WBCS,PLATELETS
  • 17. 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.
  • 18. 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,
  • 19. Treatment Steroids + Etoposide + Cyclosporine A Other considerations ATG IVIG Bone Marrow Transplant Familial Disease Non-familial: only if fail immuno-/chemo- therapy
  • 20. 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
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