5/19/17 Intern Morning Report – Fevers, Fatigue, Hepatosplenomegaly, HLH

CC: fevers, malaise, mild shortness of breath

ID: 40 yo female with no medical history presents for 1 week of fevers, malaise, and mild shortness of breath.  She also notes a 1 year history of intermittent fevers, fatigue, chills, night sweats, weight loss, and LUQ abdominal fullness.  On exam, she was noted to be febrile to 39.3 with diffuse lymphadenopathy and splenomegaly.  She was found to be pancytopenic on presentation with WBC of 1.3, Hgb of 6.5, and Plt of 79.  Further workup revealed a ferritin of 2,757, LDH of 617, and triglycerides of 251.  Autoimmune labs were negative and a CT scan demonstrated splenomegaly with multiple enhancing lymph nodes throughout.  A bone marrow biopsy demonstrated histiocytes comprising 50% of the bone marrow and the patient was diagnosed with hemophagocytic lymphohistiocytosis.

Don’t forget:

  • To meet the criteria for HLH, the patient must have the right clinical picture and 5 of the following:
    • fever > 38.5C
    • splenomegaly
    • cytopenias (at least bicytopenia with Hgb < 9, Plt < 100, or ANC < 10000)
    • hypertriglyceridemia (fasting trigs > 265) and/or hyperfibrinogenemia (fibrinogen < 150)
    • hemophagocytosis in bone marrow, spleen, lymph node, or liver
    • low or absent NK cell activity
    • ferritin > 500 ng/ML
    • elevated soluble CD25

Pearls from morning report:

  • To examine for splenomegaly, start palpation in RLQ as the spleen enlarges towards that direction.
  • The combination of pancytopenia and constitutional symptoms should lead to workup for infection (viral illness, miliary TB, fungal infections, endocarditis), HLH, hematologic malignancy, or autoimmune disease.
  • When ferritin levels are significantly elevated (in the high hundreds to thousands), causes that should come to mind include: Still’s Disease, HLH, disseminated histoplasmosis, hemochromatosis, and liver failure.
  • The workup of HLH is not complete after its diagnosis – you must evaluate for underlying precipitants/causes of HLH.

Random trivia:

HLH was first described in 1939, but more fully understood in 1952 when two siblings in a family passed away from HLH (and another sibling 6 years later).


Want to read more?

Hemophagocytic lymphohistiocytosis: review of etiologies and management (J Blood Med)


References: 

Weinzierl KP, Arber DA. The Differential Diagnosis and Bone Marrow Evaluation of New-Onset Pancytopenia. Am J Clin Pathol. 2013;139:9-29.

Berliner N, Gansner JM, Schrier SL, Rosmarin AG. Approach to the adult with unexplained pancytopenia. In: UpToDate, Post TW (Ed), UpToDate, Waltham, MA. (Accessed on May 19, 2017)

10/19/16 Resident Morning Report – FUO and Adult Onset Still’s Disease

CC: 6 days of fevers and URI symptoms

ID: 67 yo female with pernicious anemia presents with 6 days of fevers and URI symptoms and 3 weeks of nonpruritic, round, scaly rash on her forearm. Exam was notable for a temperature of 39.4C, anterior cervical lymphadenopathy, shotty inguinal lymphadenopathy, and an absence of a rash initially. After a week of inpatient evaluation, patient continued to have fevers, and was noted to have a faint, erythematous rash on her chest, back, and arms. Further studies revealed a ferritin of > 8000; patient was eventually diagnosed with adult Still’s Disease.

harrisons-fuo-algorithm

Approach to FUO Algorithm


Pearls from morning report:

  • In a patient who is >55 y.o with elevated ESR and persistent fevers, consider temporal artery biopsy.
  • Patients with adult Still’s Disease usually have fevers that spike in the afternoon or two spiking fevers in one day (“double-quotidian”).

Random trivia:

Still’s Disease was named after Sir George Frederic Still, an English pediatrician who first described juvenile rheumatoid arthritis (JRA).  The syndrome gets its name from its resemblance to the systemic form of JRA and was first described in adults by British physician, Eric Bywaters.

Also, congratulations to Mario Ho for submitting a new record high. She took care of a patient with a ferritin of 45,874 ng/mL!


Want to read more?

Fever of Unknown Origin: A Clinical Approach (The American Journal of Medicine)


References: 

Bleeker-Rovers CP, van der Meer JM. Fever of Unknown Origin. In: Kasper D, Fauci A, Hauser S, Longo D, Jameson J, Loscalzo J. eds. Harrison’s Principles of Internal Medicine, 19e. New York, NY: McGraw-Hill; 2015. http://accessmedicine.mhmedical.com/content.aspx?bookid=1130&Sectionid=79724594. Accessed October 19, 2016.