Thanks to Dr. Rachel Kyung for an outstanding presentation on idiopathic autoimmune hemolytic anemia!

Teaching Points:

• The etiology of new anemia may be broken down into the categories of decreased RBC production, increased RBC destruction, or blood loss.
• Hemolysis may be caused by intrinsic red cell defects including:
  • Enzyme deficiencies (e.g. G6PD deficiency)
  • Hemoglobin defects (sickle cell disease, thalassemias)
  • Membrane defects (hereditary spherocytosis, PNH)
• Causes of hemolysis extrinsic to red cells include MAHA (TTP, HUS, DIC, prosthetic heart valves), transfusion reaction (ABO incompatibility), PNH, infections (malaria or clostridial sepsis), or autoimmune hemolysis.
• Cold agglutinin disease is caused IgM antibodies against RBC’s which activates the classical compliment system. It may be primary (idiopathic) or secondary to infection or lymphoproliferative disorders.
• Warm agglutinin disease is characterized by IgG directed against RBC’s which results in spherocytosis on peripheral smear. Etiology may be idiopathic or secondary to infection, autoimmune disease, lymphoprolipherative disorders (CLL) or medications. Treatement includes steroids or cytotoxic drugs and splenectomy in refractory cases. Diagnosis of both cold- and warm agglutinin disease is made by a positive Coombs test.
• Click here for a recent article discussion RBC transfusion in patients with autoimmune hemolytic anemia.

Thanks to Dr. Sandy Park for an excellent case of EGPA!

Learning Points:

• Eosinophilic Granulomatosis with Polyangiitis (EGPA, formerly Churg-Strauss Syndrome) is a multisystem, small vessel vasculitis characterized by asthma, rhinosinusitis, and peripheral eosinophilia.
• EGPA may have involvement of multiple systems including pulmonary (asthma, alveolar hemorrhage), upper airway (allergic rhinitis, sinusitis), skin (nodules, maculopapular rash, leukocytoclastic vasculitis), neurologic (mononeuritis multiplex), MSK (arthralgias, myalgias), cardiac (pericarditis, heart failure), renal and GI.
• Diagnosis requires a high level of suspicion, and may be made based on the Lanham criteria or the diagnostic criteria set out by the ACR. Click here for the original 1990 article outlining EGPA diagnostic criteria.
• Severity of disease may be assessed by the Five factors score, and is influenced by age, cardiac involvement, renal insufficiency, GI involvement, and absence of ENT involvement.
• Mainstay of treatment is corticosteroids and cyclophosphamide, however new evidence indicates rituximab may be an effective steroid sparing agent in the treatment of EGPA. Click this article to read all about it!

Thank you Dr. Kristina Lee for a great morning report on a patient with a history of lupus who developed acute mental status change during hospitalization found to have communicating hydrocephalus

Learning Points:

• The overwhelming majority of causes for AMS are metabolic, toxic, and infectious
• The differential diagnosis for the neuropsychiatric manifestations of lupus is broad and includes central and peripheral processes
• For your patients on prophylactic lovenox requiring a lumbar puncture, hold the dose for 24 hours prior to procedure to avoid an epidural hematoma which can be catestrophic

Thank you to Dr. Morden for an outstanding case and presentation on multiple myeloma!

Teaching Points:

• Multiple myeloma is a neoplastic plasma cell disorder characterized by clonal proliferation of malignant plasma cells in the bone marrow, monoclonal protein in the blood or urine, and associated organ dysfunction.
• Myeloma may uncommonly present as bilateral pleural effusion, and may be due to non-malignant reasons such as heart failure or hypoproteinemia. The effusion will typically be exudative.
• Diagnostic studies include screening serum protein electrophoresis with confirmatory immunofixation, marrow biopsy, metastatic bone survey, CBC, and basic chemistries. Multiple myeloma is defined as >10% clonal bone marrow plasma cells with presence of organ impairment (anemia, hypercalcemia, renal insufficiency, or boney lesions)
• Staging of MM may be based on Durie-Salmon Criteria or ISS Criteria, with 5 year survival of Stage 1 or 2 being roughly 50% and Stage 3 being 30%.
• Click here for an excellent review article from NEJM on multiple myeloma.

Thank you Dr. Katrina DeLeon for a great morning report on Loffler’s syndrome

Teaching Points:

• Loffler’s syndrome is a disease in which eosinophils accumulate in the lung in response to a parasitic infection
  • Examples of parasites:
    • Ascarislumbricoides(roundworm)
    • Strongyloidesstercoralis(nematode)
    • Necatoramericanus(hookworm)
    • Ancylostomaduodenale(hookworm)
• Absolute eosinophil count:
  • <500 = Normal
  • 500-1500 = Mild, usually do to asthma vs. allergic rhinitis; don’t necessitate large workup
  • >1500 = Moderate and significant; warrant a workup
• Symptoms are usually mild or absent and tend to spontaneously resolve after several days or, at most, after 2-3 weeks.
  • Fever, malaise, cough, wheezing, and dyspnea are the most common symptoms. Less commonly, the patient may present with myalgia, anorexia, and urticaria.
  • Social and travel history should be carefully elicited to identify risk factors for exposure to parasites.

Thanks to Dr. Erica Tate for a great presentation of Dermatomyositis!

Learning Points:

• Diagnostic criteria: symmetric proximal muscle weakness, elevation of muscle enzyme levels, abnormal EMG results, muscle biopsy abnormalities and typical skin rash
• Pathogenesis: complement mediated vasculopathy of the small vessels in muscle tissue resulting in ischemia and vessel damage
• Clinical presentation: classically presents with heliotrope rash, gottron’s papules and other dermatologic manifestations prior to onset of proximal muscle weakness. Can also affect cardiovascular, pulmonary and GI systems
• Treatment: gold standard is initiation of corticosteroids followed by replacement with steroid-sparing agents such as Methotrexate, Mycophenolate mofetil and Azathioprine. In refractory cases, other treatment options include IVIG, Cyclosporin and  Rituximab

Enjoy this great article on Dermatomyositis for further learning!