11 Apr 2021

11/25/20: Acute Promyelocytic Leukemia (APML)

PGY2 Sami Dwabe presented a case of a 55-year-old male with history of Hodgkin Lymphoma (age 12, s/p chemo/XRT), pancreatic neuroendocrine tumor s/p distal pancreatectomy (2017) with no evidence of disease, rectal adenocarcinoma (2018) s/p resection/adjuvant FOLFOX x6 with no evidence of disease, bioprosthetic AV, history of PE (2017) now off Xarelto, and DM type II presents with 2 weeks of progressive shortness of breath and fatigue.  Patient was recently on a trip, feeling in normal state of health until he noticed symptoms ~2 weeks ago with shortness of breath, fatigue, worse with exertion.  Home medications include Atorvastatin 40mg qday, Cholestyramine 4g, Dicyclomine 10mg BID, Glipizide 10mg qday, Levothyroxine 150mcg qday, Januvia 100mg qday, and Lexapro 10mg qday.  No sick contacts but recent travel.  No other drug or tobacco use.

Vitals: T 36.4,HR 96, RR 27, BP 103/56, O2 sat 99% on 8L NC

Physical Exam significant for A&Ox3, 2/6 systolic murmur, tachypneic, crackles bilaterally, decreased breath sounds at bases, no edema in b/l LE, +BRBPR.

CBC: WBC 31.14> Hb 7.3/Hct 22.5 <Plt 63 (MCV 82.1, RDW 23.4%)

CMP:

Na 131/K 5.2/Cl 95/HCO3 22/BUN 45/Cr 3.13 <Glucose 166 (Baseline Cr: 1.16 2 months)

ALP 271>T.protein 4.6/Albumin 2.1/AST 23/ALT 8/Tbili 1.7/Dbili 1.6

Ca 7.8, Mg 1.6, Phos 3.2

Coags: PT 23.2, INR 2, PTT 45.7

Stat ABG, CXR and CTPA was obtained:

  • ABG: 7/43/33/263/22, FiO2 100%
  • CXR showed small L and trace R pleural effusions, mild pulmonary vascular congestion and L>R lung base airspace opacity may reflect atelectasis.
  • CTPA showed R lower lobe PE, consolidative opacities as well as groundglass and solid nodules within the upper lobes and increasing pleural effusions with increased adjacent atelectasis.

Infection workup was initiated while pending manual differentiation and peripheral smear:

  • Blood culture: Klebsiella pneumoniae x2
  • COVID-19 not detected
  • Urine culture negative
  • Sputum culture: 2+ normal upper respiratory flora

Heme workup revealed:

  • Retic %: 0.9%, Abs Retic 0.027
  • Uric acid: 4.7 (normal: 3.4 – 7)
  • Iron panel: Iron 64 (59 – 158), TIBC 120 (normal 250 – 425), Iron Sat 53 (normal 20 – 50), Ferritin 1207 (30 – 400)
  • LDH: 552 (normal 135 – 225)
  • Haptoglobin: 126 (normal 30 – 200)
  • Fibrinogen: 248
  • D-Dimer: >10,000 (normal <249)
  • Manual Diff: 7.7% Segs, 0.9% Band, 0.9% Lymph, 89% Blasts
  • Peripheral smear: numerous blasts with Auer rods

Treatment: Patient initially admitted to Oncology service for concern of infection vs VTE. However, once blood smear was seen, patient was transferred to the Heme service and started on ATRA. Given worsening AKI and anuric, patient was started on HD.

Teaching Points:

“Can’t Miss” Hematologic Diagnoses

ProcessSignificant findingsLabs
Destructive Process associated with multiple cytopenias  
Hemolytic-Uremic syndrome (HUS)Hemolysis, Schistocytes, Renal failurePeripheral smear, CH50
Thrombotic Thrombocytopenic Purpura (TTP)Hemolysis, Schistocytes, pentad of fever, anemia, thrombocytopenia, renal and neuro changes (FAT RN)Peripheral smear, ADAMSTS13
Disseminated intravascular coagulation (DIC)Schistocytes and abnormal coagulation test resultsPT, aPTT, Fibrinogen, Plt
Hemophagocytic lymphohistiocytosis (HLH)Hepatomegaly and splenomegaly, elevated ferritinTG, NK cell activity, Ferritin, CD25
Infiltrative processes associated with multiple cytopenias  
Acute promyelocytic leukemia (APML, can be leukopenic or lymphocytosis) Microgranular variant – no auer rods, high WBCOften associated with DICPeripheral smear, flow cytometry
Other cancers: AML, ALL, MMRapid proliferation and infiltration of the BM which leads to pancytopenia 
Destructive processes associated with individual cytopenias  
Heparin induced thrombocytopenia (HIT)Isolated thrombocytopenia4T score, HIT screen
Immune thrombocytopenic purpura (ITP)Isolated thrombocytopenia 
Paroxysmal nocturnal hemoglobinuria (PNH)Isolated anemia (from hemolysis)CD55, CD59
Processes often in the heme differential diagnosis that may be indirectly associated with cytopenias  
Antiphospholipid antibody syndrome (APS)Multiorgan involvement, profound inflammatory stateLupus anticoagulant
PorphyriaNeurovisceral symptoms 

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