Ambulatory Report Pearls – LAD, Pancytopenia, Lupus – 6.29.16

Thanks to Neha Gupta for presenting a case of 45 yo M h/o PTSD & knee/ankle pain p/w initially with profuse watery diarrhea and rash which improved after several outpatient visits, however who ultimately developed persistent fevers, weight loss, night sweats, diffuse lymphadenopathy, and pancytopenia representing most likely a Lupus Flare! (what a crazy twist at the end…I bet you thought I was gonna say Lymphoma!)
  • Remember the Diagnostic Criteria for Lupus
    • Mucocutaneous
      • Malar Rash – with scale and induration
      • Discoid Rash – particularly on scalp and ears
      • Photosensitivity – look in sunexposed areas; erythematous and stays red (can be annular or psoriaform)
      • Oral Ulcers – can be on buccal mucosa, hard/soft palate, and often are painless
    • Organs
      • Arthritis – have ligimentous laxity, and see normal bones on plain film; often have migratory arthritis
      • Serositis – pericarditis, pleuritis, keratinitis
      • Renal Disease
      • Neurologic d/o – seizure, psychosis
    • Labs
      • Hematologic
        • hemolytic anemia
        • Leukopenia (< 4)
        • Lymphopenia (< 1.5)
        • Thrombocytopenia (<100)
      • Immunologic – positive anti-phospholipid ab, anti-dsDNA, antiSm
      • +ANA (titer matters)
  • Pancytopenia
    • APPROACH = Depends on the Marrow
      • Pancytopenia w/ HYPOcellular marrow – like a garden that died
        • acquired: radiation, drugs, viruses (EBV, CMV, HIV, Hepatitis), immune diseases (SLE), pregnancy
        • toxin (EtOH) & Meds (Bactrim, chemo, immunosuppression)
        • infection (Mycobacteria, TB)
        • inflammation
        • hypothyroidism
        • inherited: Fanconi’s anemia, Dyskeratosis congenita, Shwachman-Diamond, Down syndrome, Amegakaryocytic thrombocytopenia
      • Pancytopenia w/ CELLULAR marrow – like a garden with no growth
        • intrinsic defect (PNH)
        • MDS
        • leukemia
        • suppression
      • Pancytopenia w/ marrow replacement – like a garden overtaken by weeds
        • myelofibrosis
        • myelophthisis: means that marrow has been taken over by too much of something! Tumor, fibrosis, granulomas
          • leukoerythroblastic changes on smear: tear drops, nucleated RBCs, immature granulocyte precursors
        • lymphoma
      • Sequestration
        • splenomegaly
    • W/u (consider sending these, doesn’t mean definitely send everything the second you see all cell lines declining)
      • Is a Bone Marrow Biopsy needed?
      • Smear & Iron Studies
      • B12, MMA, Folate, TSH, Hepatitis Serologies,
      • EBV, Parvorius as appropriate
      • ANA (if concerned about an immune mediated process)
      • SPEP/UPEP, Gamma Gap
      • Think about RPR for syphillis
      • Place PPD or send Quantiferon if RFs for TB
      • Abd u/s (if c/f sequestration to assess for splenomegaly)
      • CD 55 and 59 for PNH (only IF the story fits!!)
    • Review Article

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