Bone Marrow Disorders (Holman) Flashcards
What is the first major distinction that occurs in the life of a cell that began as a pluripotent stem cell?
differentiation to myeloid or lymphoid lineage
Which of the following cannot be determined about red blood cells via peripheral blood smear? A. cell number B. morphology C. polychromasia D. left-shift E. rouleaux
D. Left-shift means that there are more than are expected of the earlier myeloid precursors. This information can be obtained via bone marrow aspiration.
polychromasia is a disorder where there is an abnormally high number of red blood cells found in the bloodstream as a result of being prematurely released from the bone marrow during blood formation. These cells are often shades of grayish blue and can be seen on blood smear.
rouleaux are stacks or aggregations of red blood cells (RBCs) which form because of the unique discoid shape of the cells. This is in indicator of disease.
Which of the following cannot be determined from a bone marrow aspirate? A. dyspoiesis B. number of megakaryocytes C. myeloid:erythroid ratio D. number and morphology of lymphocytes E. evaluation for metastatic tumor
B. Megakaryocytes tend to get pushed to the edge of an aspirate and are not well represented. They are much easier to find in a biopsy.
With a bone marrow biopsy you can also determine #cells:fat and confirm the myeloid to erythroid ratio.
Because the architecture of the cells remains intact you can also see fibrosis and aggregates of lymphocytes, blasts and tumor cells.
When does mitosis cease in a granulocytic cell?
At the myelocyte - metamyleocyte transition. At this point cells stop dividing and just mature.
the _____ is composed of neutrophils that are counted in a peripheral blood smear whereas the _____ contain neutrophils that remain adhered to the sides of the vessels until they are mobilized (ie, by stress or infection)
circulating granulocyte pool; marginating granulocyte pool
where does nuclear extrusion of red blood cells occur?
in the bone marrow, just before the cells are released into the blood
a disease in which stem cells are unable to generate mature blood cells, causing a deficiency of all three blood cell types (pancytopenia): red blood cells (anemia), white blood cells (leukopenia), and platelets (thrombocytopenia)
aplastic anemia
disorder in which there are plenty of erythroid precursors but few granulocyte precursors (ie, the body makes an antibody to its neutrophil precursors)
granulocytic aplasia
ineffective hematopoiesis, in which the marrow is packed with plenty of granulocyte precursors but they are not able to mature and get out into the blood stream, may be caused by what deficiency?
vitamin b12
gum inflammation is a common clinical presentation of _____.
neutropenia
an increase in the number of neutrophils, resulting in demargination, mobilization of stores, and increased production is known as reactive _____.
neutrophilia
extreme leukocytosis in response to stress or infection that may resemble leukemia; increased immature cells are seen
leukemoid reaction
____ leukemia is mostly associated with blast formation and ____ leukemia is associated with differentiated cells
acute; chronic
malignant clonal neoplasm of any myeloid lineage cell characterized by the accumulation of myeloblasts in the blood and bone marrow (at least 20%)
acute myeloid leukemia (AML)
All of the following are markers of myeloid cell lineage EXCEPT: A. CD117 B. CD13 C. CD19 D. CD33 E. Auer rods
C. This is a B cell marker.
subtype of AML characterized by either microgranules or hypergranules with a butterfly shaped nucleus and multiple auer rods
acute promyelocytic leukemia; caused by a t(15;17) translocation (KNOW THAT), that leads to constituous activation of a tyrosine kinase protein leading to proliferative blast formation and eventually DIC (not good)
Treatment with all trans retinoic acid (ATRA) to allow cells to progress past the blast stage
subtype of AML characterized by abundant blast cell cytoplasm and may exhibit extramedullary presentation (ie, skin, gums, CNS)
acute myelomonocytic leukemia
subtype of AML characterized by blebbing cytoplasm off the side of the cell
acute megakaryoblastic leukemia
malignant neoplasm of lymphoid stem cells, may show markedly increased blasts and is the most common cancer in children age 1-17
acute lymphoblatsic leukemia (ALL)
classified by flow cytometric markers, with most being precursor B cells; good prognosis in children (85% cure rate)
What do the presence of auer rods and a positive MPO tell you, without the need for flow cytometry?
indicative of AML
leukemia that is rare in children and is characterized by the presence of mostly differentiated cells (e.g., neutrophils and granulocytic precursors), left shift, and invariable basophilia
chronic myeloid leukemia
etiology: t(9;22)
presentation: fatigue and weight loss, sometimes splenomegaly that gets worse with progression of disease
drug developed to treat CML, which fits into the binding pocket of the fused constiutively active bcr/abl protein, interrupting phosphorylation of downstream substrates
imatinib (Gleevac); expanded the life expectancy from 5 years to normal (!!)
clonal B-cell neoplasm which expresses mature B-cell antigen, monotypic lambda or kappa light chains, and aberrantly express the T-cell marker CD5; “smudge” cells apparent on blood smear
chronic lymphocytic leukemia (CLL)
presentation: > 60 years old, most often in males, generalized lymphadenopathy in 50-60% of cases, widespread lymphocytosis, immune dysregulation
name given to clonal myeloid stem cell disorders, which are characterized by cytopenia, ineffective hematopoiesis, and dyspoiesis with an increased risk of progression to AML (1 in 3)
myelodysplastic syndromes (MDS)
presenation: elderly patients >60, 50% are asymptomatic, the other 50% present with fatigue, hemorrhage, infections; variable but generally poor prognosis
disorder whose blood smear shows dimorphic red cell population, hypercellular marrow, dyspoiesis, and ringsideroblasts
MDS
etiology: mostly trisomies and deletions
Which of these is NOT a type of chronic myeloproliferative neoplasm? A. Primary myelofibrosis B. MDS C. Essential thrombocythemia D. CML E. Polycythemia Vera
B.
A mutation in this gene is the hallmark of all chronic meyloproliferative neoplasms (excluding CML, which is characterized by a mutation in the BCR/ABL gene)
JAK2 gene
All of the following are characteristic of essential thrombocythemia EXCEPT:
A. Most commonly appears in children
B. Splenomegaly is commonly seen
C. May present with either thrombosis or hemorrhage
D. Indolent disorder with long survival
E. Rarely transforms to AML
A. This is an adult disease that most commonly affects people age 50-60
All of the following are characteristic of essential thrombocythemia EXCEPT:
A. Usually an adult disease
B. Splenomegaly is commonly seen due to extramedullary hematopoiesis
C. May present with either thrombosis or hemorrhage
D. It is an indolent disorder with long survival that rarely transforms to AML
E. Characterized by large atypical platelets and megakaryocytes
B. This is a feature of primary myelofibrosis.
All of the following are characteristic of primary myelofibrosis EXCEPT:
A. Most common in middle age and elderly
B. Associated with poor prognosis with up to 20% developing AML
C. Teardrop red blood cells and clustered megakaryocytes
D. Reactive deposition of fibrous connective tissue in the bone marrow
E. Leukoerythroblastic reaction (ie, circulating bone marrow components in the blood stream) is specific for this disease
E. This reaction may be seen in response to normal stress situations as well (e.g., newborns display this reaction as well)
diseases characterized by clonal proliferation of terminally differentiated B cells capable of producing Ig and producing large amounts of M protein
plasma cell neoplasms
examples: MGUS, plasmacytoma, plasma cell myeloma
most asymptomatic plasma cell neoplams marked by a small increase in monoclonal (M) protein without overt disease
monoclonal gammopathy of undetermined significance (MGUS)
About 25% of patients progress to plasma cell neoplams over 20 years
disease marked by solitary bone lesion or extraosseus lesion, usually lung, pharynx or nasal sinuses; patients do not often show M spike
plasmacytoma
bone marrow based disease with involvement of the skeleton at multiple sites and shows significant M spike
plasma cell myeloma
pneumonic for symptoms, CRAB = Calcemia Renal insufficiency Anemia Bony lytic lesions
