Myeloproliferative neoplasm differential diagnosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mohamad Alkateb, MBBCh [2] Shyam Patel [3]

Overview

Myeloproliferative neoplasm must be differentiated from acute lymphoblastic leukemia, acute myelogenous leukemia, chronic myelogenous leukemia, essential thrombocytosis, hypereosinophilic syndrome, non-Hodgkin lymphoma, primary myelofibrosis, secondary thrombocytosis, splenomegaly, systemic mastocytosis, and waldenstrom macroglobulinemia.

Differentiating Myeloproliferative neoplasm from other Diseases

Characteristic Causes Laboratory abnormalities Physical examination Therapy Other associations
Myeloproliferative neoplasm
  • JAK2 mutation
  • CALR mutation
  • MPL mutation
  • BCR-ABL translocation
  • CSF3R mutation
  • SETBP1 mutation
  • PDGFRA or PDGFRB rearrangement
  • Splenomegaly
  • Hepatomegaly
  • Evidence of infection
  • Pallor
  • Ruxolitinib
  • Hydroxyurea
  • Anagrelide
  • Imatinib
  • Midostaurin
  • Stem cell transplant
  • Variable features based on the subtype of myeloproliferative neoplasm
Myelodysplastic syndrome
  • Prior exposure to alkylating agents
  • Prior exposure to topoisomerase II inhibitors
  • Age-related changes in hematopoietic stem cells
  • Deletion of chromosome 5q or 7
  • Gain of chromosome 8
  • Lenalidomide
  • Decitabine
  • Azacitidine
  • Erythropoiesis-stimulating agents (ESAs)
  • Granulocyte colony-stimulating factor (G-CSF)
  • Transfusion support
  • Stem cell transplant for high-risk myelodysplastic syndrome
  • Age-related changes in the bone marrow contribute to myelodysplastic syndrome
Acute myeloid leukemia
  • Chromosomal instability
  • Sporadic mutations
  • Prior exposure to benzene
  • Prior exposure to alkylating agents
  • Prior exposure to topoisomerase II inhibitors
  • Germline RUNX1 mutation
  • Pyrexia
  • Evidence of infection
  • Pallor
  • Mucosal bleeding
  • Bruising
  • Cytarabine
  • Anthracycline
  • Enasidenib
  • Liposomal daunorubicin plus cytarabine
  • Gemtuzumab ozogamycin
  • Midostaurin
  • Stem cell transplant
  • Variable prognosis based on cytogenetic and molecular profile
  • Four new FDA-approved therapies became available in 2017
Acute lymphoblastic leukemia
  • Chromosomal instability
  • Sporadic mutations
  • Anemia
  • Thrombocytopenia
  • Neutropenia
  • Elevated LDH
  • Elevated uric acid
  • Elevated phosphorus
  • Elevated potassium
  • Low calcium
  • Greater than 20% lymphoblasts on bone marrow aspirate
  • Neurologic deficits
  • Pallor
  • Lymphadenopathy
  • HyperCVAD (cyclophosphamide, vincristine, doxorubicin, dexamethasone)[2]
  • R-HyperCVAD (inclusion of rituximab)
  • Peg-asparaginase
  • Intrathecal methotrexate
  • Intrathecal cytarabine
  • Blinatumomab (bispecific T cell engager)
  • Inotuzumab ozogamycin (anti-CD22 antibody)
  • Tisagenlecleucel (chimeric antigen receptor T (CAR-T) cell therapy)
  • Stem cell transplant
  • Sanctuary sites include the central nervous system (CNS) and testes[3]
Waldenstrom's macroglobulinemia
  • MYD88 mutation
  • Lymphoplasmacytic cell proliferation
  • Elevated immunoglobulin M (IgM) paraprotein
  • Presence of M-spike on protein electrophoresis
  • Elevated serum free light chains (kappa and lambda)
  • Increased serum viscosity
  • Hepatomegaly
  • Splenomegaly
  • Retinal vascular dilation and thrombosis
  • Decreased visual acuity
  • Headache
  • MYD88 mutation testing is standard-of-care
  • Plasmapheresis should be initiated if symptoms of hyperviscosity are present
  • Typically does not require stem cell transplant
Lymphoproliferative disorder[4]
  • Elevated lymphocyte count with presence of clonality
  • Anemia
  • Thrombocytopenia
  • Neutropenia
  • Variable based on the etiology
  • Cytotoxic chemotherapy
  • Antiviral agents
  • Biologic therapy with anti-CD20 monoclonal antibodies
  • Tapering immunosuppressive medications (for post-transplant lymphoproliferative disorder)
  • Can be due to a variety of causes
  • Variable prognosis


References

  1. Döhner H, Estey E, Grimwade D, Amadori S, Appelbaum FR, Büchner T; et al. (2017). "Diagnosis and management of AML in adults: 2017 ELN recommendations from an international expert panel". Blood. 129 (4): 424–447. doi:10.1182/blood-2016-08-733196. PMC 5291965. PMID 27895058.
  2. Terwilliger T, Abdul-Hay M (2017). "Acute lymphoblastic leukemia: a comprehensive review and 2017 update". Blood Cancer J. 7 (6): e577. doi:10.1038/bcj.2017.53. PMC 5520400. PMID 28665419.
  3. Inaba H, Greaves M, Mullighan CG (2013). "Acute lymphoblastic leukaemia". Lancet. 381 (9881): 1943–55. doi:10.1016/S0140-6736(12)62187-4. PMC 3816716. PMID 23523389.
  4. Kipps TJ, Stevenson FK, Wu CJ, Croce CM, Packham G, Wierda WG; et al. (2017). "Chronic lymphocytic leukaemia". Nat Rev Dis Primers. 3: 16096. doi:10.1038/nrdp.2016.96. PMC 5336551. PMID 28102226.

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