Heme malignancies Flashcards
What is characterized by asymptomatic and incidental finding of abnormal blood counts, with occasional cytopenia (fatigue, infection, bleeding, fever, weight loss, general debility)?
myelodysplastic syndrome
What makes someone at risk for myelodysplastic syndrome?
prior exposure to cytotoxic chemo and radiation, >60
What is myelodysplastic syndrome?
type of leukemia that is a precursor to acute leukemia –> buildup of IMMATURE cells w a “constellation of cytopenias w/ hypercellular marrow, morphologic dysplasia”
increase in bone marrow blasts (more aggressive)
can have presence of defining genetic alterations!
What on a PE would signify myelodysplastic syndrome?
splenomegaly, pallor, bleeding, infection
What on a CBC would signify myelodysplastic syndrome?
anemia w/ normal or increased MCV, left shift and WBC/platelets normal or reduced
What on a peripheral blood smear would indicate myelodysplastic syndrome?
macro-ovalocytes, neutrophils w/ morphologic abnormalities (# of granules, Pelger-Huet abnormality), small # of promyelocytes or blasts, platelets
What’s key about the blast percentage in myelodysplastic syndrome?
<20% blasts, if more, then think AML
What on a bone marrow biopsy indicates myelodysplastic syndrome?
hypercellular (LOTS of cells), erythroid hyperplasia, abnormal erythropoiesis
Prussian ringed blue stain = ringed sideroblasts
Myeloid series often left shifted w/ increases in blasts, dwarf megakaryocytes w/ unilobed nucleus
How can you craft a diagnosis for myelodysplastic syndrome?
with genetic abnormalities! Generally chromosome 5 & 7
How can you treat myelodysplastic syndrome?
low risk: erythropoietic growth factors, w/ chromosome abnormalitiy: lenalidomide, immunosuppressive therapy (anti-thymocyte globulin)
high: hypomethylating agents (azacitidine or decitabine), bone marrow transplant
consider transfusions like RBC for anemia, platelets for bleeding
Deferasirox to eliminate iron buildup, abx to prevent infections
What is indicated by a chronic phase sometimes followed by an acute blast phase involving fatigue, night sweats, low-grade fevers, abdominal fullness w/ splenomegaly and with accelerated disease involving fever, bone pain, and splenomegaly?
chronic myeloid leukemia
In who is chronic myeloid leukemia more likely?
middle age
What causes chronic myeloid leukemia?
overproduction of myeloid cells – Philadelphia chromosome of a fusion gene bcr/abl that induces leukemia!
What on a PE could indicate chronic myeloid leukemia?
splenomegaly + sternal tenderness
What on a CBC could indicate chronic myeloid leukemia?
elevated WBC, left shift, <5% blasts, basophilia and eosinophilia, with normalish platelets (Hct, Hg, RBCs)
What type of test is essential for chronic myeloid leukemia?
bone marrow biopsy
What on a bone marrow biopsy could indicate chronic myeloid leukemia?
karotype and hypercellular w/ left-shifted myelopoiesis
What’s the hallmark of chronic myeloid leukemia?
bcr/abl gene with Philadelphia chromosome, detected by PCR
With chronic myeloid leukemia progression to blast phase, what should indicate that?
> 20% of blast M cells and progressive anemia (more pronounced) + thrombocytopenia
How should you treat chronic myeloid leukemia?
extreme hyperleukocytosis w/ priapism (resp distress, visual blurring, AMS) - emergent leukapheresis w/ myelosuppressive therapy
chronic phase CML: tyrosine kinase inhibs (imatinib)
advanced stage (blast crisis): tyrosine kinase inhib alone or combo w/ myelosuppressive therapy
What does fatigue or lymphadenopathy with 50% having hepatomegaly or splenomegaly indicate?
chronic lymphocytic leukemia
In who is chronic lymphocytic leukemia more likely?
> 50y, m: 70
What causes chronic lymphocytic leukemia?
malignancy of B lymphocytes with immunosuppression, bone marrow failure, organ infiltration
What can indicate chronic lymphocytic leukemia?
isolated lymphocytosis!
WBC elevated, Hct and platelets normal initially
bone marrow = small but mature lymphocytes
smudge = cell remnants from fragile cells, lymph nodes infiltrated
What are indications for treatment with chronic lymphocytic leukemia?
progressive fatigue, symptomatic lymphadenopathy, anemia, thrombocytopenia
How do you treat chronic lymphocytic leukemia if it is indicated?
targeted biologic therapy – BTK inhibitor (ibrutinib, acalabrutinib, zanubrutinib)
OR
venetoclax w/ anti-CD20 antibody therapy (obinutuzumab)
for super severe = allogeneic transplantation which is high risk but needed for super severe
What is indicated by bleeding (such as gingival, epistaxis, menorrhagia, from thrombocytopenia) and infection (like cellulitis, pneumonia, perirectal infections, fungal, death from neutropenia) that risks rise with <500 neutrophil and can have gum hypertrophy, bone/joint pain, and severe illness with hyperleukocytosis w/ headache, confusion, dyspnea?
acute myeloid leukemia
In who is acute myeloid leukemia common?
MC malignancy of adults ~60y and with down syndrome
What is acute myeloid leukemia caused by?
malignancy of myeloblasts
What in a PE does acute myeloid leukemia indicate?
pale, purpura, petechiae, stomatitis, gum hypertrophy, rectal fissures, enlargement of liver, spleen, lymph nodes, bone tenderness of sternum, tibia, femur
What are some diagnosis features of acute myeloid leukemia?
pancytopenia w/ circulating blasts, and bone marrow = hypercellular and dominated by blasts (>20%), hyperuricemia
What is key in differential diagnosis of acute myeloid leukemia?
Auer rod (eosinophilic needle-like inclusion in cytoplasm)
How can you treat acute myeloid leukemia?
curative intent – anthracycline (danurubicin or idarubicin) + cytarabine (alone or in combo w/ other agents)
non curative – (>75y or significant comorbidities) newer treatments
How can one differentiate acute lymphoblastic leukemia?
similar symptoms to AML but “bulky disease”, some lymphadenopathy, hepatosplenomegaly
In who is acute lymphoblastic leukemia common?
MC acute leukemia of childhood 3-7y, down syndrome
What causes acute lymphoblastic leukemia?
malignancy of precursor B or T lymphocytes
What differentiates acute lymphoblastic leukemia?
mediastinal mass visible on CXR
What should you remember about treatment for ALL?
young adults have better outcomes when treated like peds protocol, with older patients going straight to allogeneic transplantation
How do you treat acute lymphoblastic leukemia?
combo chemi including daunrubicin, vincristine, prednisone, asparaginase
if they have the philadelphia chromosome, add a tyrosine kinase inhibitor (dasatinib or ponatinib)!
How should you treat acute lymphoblastic leukemia after remission?
high dose chemo/additional cycles + stem cell transplantation
What does enlarged lymph nodes indicate with “B symptoms” being fever, drenching night sweats, weight loss >10% body weight?
non-hodgkin’s lymphoma
What causes non-hodgkin’s lymphoma?
proliferation of B (most) or T cells, spreading non-linear, skipping around to extranodal sites
What are some notable things on a non-hodgkin’s lymphoma PE?
lymphadenopathy isolated or diffuse, extranodal sites of disease may be found (skin, GI tract, liver, bone marrow)
Burkitt=abdominal pain/fullness
How can you help diagnose non-hodgkin’s lymphoma?
tissue biopsy for accuracy and classification
high grade –> CSF, serum LD - useful prognostic
How can you treat indolent non-hodgkin’s lymphoma?
local irradiation w/ cure intent
disseminated disease - not curable, only offered when symptoms develop or tumor bulk
How do you treat aggressive non-hodgkin’s lymphoma?
R-CHOP
What is characterized by a painless mass, commonly in the neck, w/fever, weight loss, drenching night sweats, general pruritus, when drinking, can become painful?
hodgkin’s lymphoma
What puts someone at risk for hodgkin’s lymphoma?
EBV virus, 20s and >50
What causes hodgkin’s lymphoma?
unknown but spreads contiguously, cancer of germinal center B cells
How do you diagnose hodgkin’s lymphoma?
lymph node biopsy required for diagnosis – Reed-Sternberg cells are key!
How can you treat hodgkin’s lymphoma?
chemotherapy with dosing based on risk, with either low risk or high risk, ABVD
What is characterized by CRAB - high levels of calcium, decreased renal function, low amount of RBCs, bone damage, and can have hyperviscosity syndrome?
plasma cell myeloma/multiple myeloma
What causes plasma cell myeloma/multiple myeloma?
proliferation of plasma cells in bone which makes them prone to infection, so vaccinate!
What on the PE is key for plasma cell myeloma/multiple myeloma?
pallor, bone tenderness, soft tissue masses, neurologic signs w/ neuropathy or spinal cord compression, fever w/ infection, oliguria
What’s key in labs for plasma cell myeloma/multiple myeloma?
Bence Jones proteinuria, anemia (rouleaux formation), lytic lesions in radiograph w/ diagnosis involving analysis of serum protein electrophoresis and free light chains + bone marrow biopsy
How can you treat plasma cell myeloma/multiple myeloma?
chemo w/ immunomodulatory agent
