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
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	Dysplasia in at least 10% of cells of at least 1 cell line Low white blood cell count Anemia Neutropenia Thrombocytopenia	Mucosal bleeding Petechiae Ecchymoses Evidence of infection Pallor	Lenalidomide Decitabine Azacitidine Erythropoiesis-stimulating agents (ESAs) Granulocyte colony-stimulating factor (G-CSF) Transfusion support	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	Anemia Thrombocytopenia Neutropenia Elevated LDH Elevated uric acid Elevated phosphorus Elevated potassium Low calcium Greater than 20% myeloblasts on bone marrow aspirate	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) 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
Chronic myeloid leukemia	Translocation between chromosomes 9 and 22 Creation of BCR-Abl gene product	Elevated white blood cell count Presence of white blood cell precursors at various stages of maturation Presence of excess metamyelocytes, basophils, eosinophils, and band cells	Splenomegaly Abdominal tenderness Pallor Evidence of infection	Imatinib Nilotinib Dasatinib Bosutinib Ponatinib Omacetaxine	High response rate to tyrosine kinase inhibitors Risk for development of T315I kinase domain mutation Typically does not require stem cell transplant Three phases include chronic, accelerated, and blast phase
Chronic lymphocytic leukemia	Chromosomal instability Sporadic mutations Infections	Elevated absolute lymphocyte count (in all stages) Presence of >5000 clonal B cells per microliter in peripheral blood Anemia (in Rai stage III) Thrombocytopenia (in Rai stage IV)	Lymph node enlargement in Rai stage I Splenomegaly in Rai stage II Hepatomegaly in Rai stage II Pallor Bleeding	Fludarabine Cyclophosphamide Rituximab Obinutuzumab Ofatumumab Ibrutinib Venetoclax	Associated with autoimmune hemolytic anemia, which occurs in 10-25% of patients with CLL Associated with immune thrombocytopenia purpura Associated with pure red cell aplasia Treatment with corticosteroids or anti-leukemic therapy will correct the autoimmune complications of CLL