U A M S - I M

ACD IMAGE 07/07

WHAT IS THE DIAGNOSIS?

July 13 May 14

PREVALENCE OF MALIGNANCE IN LUNG NODULES ACCORDING TO SIZE

• > 20 mm = ≥ 65%• > 30 mm = 93%• LUNG MASS (>3 cm lung opacity) is cancer until

proven otherwise >>> Biopsy

Wahidi MM, Govert JA, Goudar RK, et al. Evidence for the treatment of patients with pulmonary nodules: when is itlung cancer? An ACCP evidence-based clinical practiceguideline (2nd edition). Chest 2007; 132:94S–107S

DIAGNOSTIC WORK UP FOR SOLITARY PULMONARY NODULE

Gould MK et al. Evaluation of individuals with pulmonary nodules: when is it lung cancer? diagnosis and management of lung cancer, 3rd ed: ACCP evidence-based clinical practice guidelines. Chest 2013 ; 143: 5: e93S - e120S .

RAHUL RAV ILL A

PGY-3 IM UAMS

ACD TEACHING POINTS- 7/7/2014

HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS

• Syndrome of excessive inflammation and tissue destruction.• Macrophages - hyperactivity, NK

cells and Cytotoxic lymphocytes- hypo activity.• Ifn-ƴ, TNF-α, IL-6,12,10 and

soluble IL-2 receptor (CD 25) are elevated.• Predominantly pediatric illness

with incidence of 1 in 100000.

CLASSIFICATION

• Familial HLH (FLH)- Autosomal recessive. Mutations in components of perforin mediated cytotoxicity

• Chediak-Higashi Syndrome, X-linked lymphoproliferative disease.

• Secondary-• Infection- EBV, CMV, Parvo, HSV, VZV, Measles, HHV-8,

H1N1, HIV, Brucella, Gram negative bacteria, Tuberculosis, Parasites, Fungi.

• Malignancy- Leukemia, Lymphoma (NK cell, T cell, ALCL)• Autoimmune- SLE, Juvenile idiopathic arthritis, Still’s

disease.• Immunodeficiency- HIV, liver and kidney transplant

DIAGNOSTIC CRITERIA

At least 5 of the following1.Fever- >38.5C2.Splenomegaly3.Peripheral blood cytopenia- Hg <9, platelet <

100000, ANC < 10004.Hypertriglyceridemia- >265mg/dl and/or

Fibrinogen <150mg/dl5.Hemophagocytosis in bone marrow, spleen,

lymph node or liver6.Low or absent NK cell activity7.Ferritin >500ng/ml (>3000 more suggestive)8.Elevated soluble CD25.

MANAGEMENT

• Supportive care• Early recognition• Treatment- • Dexamethasone- 10mg/m2 for 2 weeks,

tapered to 5 and 2.5 every 2 weeks, week 7- 1.25 and week 8 to zero

• Etoposide- VP-16- 150mg/m2 for adults (dose to be adjusted for renal clearance)

• Cyclosporin• Alemtuzumab• Intrathecal methotrexate and hydrocortisone• Allogenic HCT

IBD AND HLH

• Few case reports• Most cases associated with CMV colitis• Few cases associated with Remicade (infliximab)• Idiopathic cases improved with

colectomy/management of IBD.

References• Uptodate• Pubmed