WBC disorders Flashcards
Causes of painless lymphadenopathy
- Chronic inflammation
- Metastatic carcinoma
- Lymphoma
Treatment for follicular lymphoma
Rituximab (anti-CD20 antibody)
*Reserved for symptomatic patients
Which of the myeloproliferative disorders is not associated with increased risk for hyperuricemia or gout?
Essential thrombocythemia
Polycythemia vera is associated with a ________ mutation.
JAK2 kinase
What is the blood smear characteristic of mycosis fungoides?
Lymphocytes with cerebriform nuclei (Sezary cells)
Why is there an increased risk for infection in multiple myeloma?
monoclonal antibody lacks antigenic diversity; infection is the most common cause of death in multiple myeloma
Which neoplasm is associated with reactive polycythemia?
Renal cell carcinoma
most common age of patients with AML
50-60
Which markers are positive in Hodgkin lymphoma?
CD15 AND CD30
Which type of myeloproliferative disorder rarely progresses to marrow fibrosis or acute leukemia?
Essential thrombocythemia
*Platelets are just blebs of cytoplasm from megakaryocytes
What is the cause of marrow fibrosis in myelofibrosis?
Megakaryocytes produce excess platelet-derived growth factor (PDGF) cuasing marrow fibrosis
Causes of eosinophilia
- Allergic reactions (type I hypersensitivity)
- Parasitic infections
- Hodgkins lymphoma
A negative monospot test suggests ______ as possible cause of mono.
CMV
T-ALL is characterized by which markers?
CD2 to CD8
*The blasts do NOT express CD10
Adult T- cell leukemia is associated with HTLV-1 and is most commonly seen in which countries?
Japan and the Caribbean
- Lacunar cells
- Enlarging cervical or mediastinal lymph node
Nodular sclerosis (Hodgkin lymphoma)
BCl2 expression in follicles. Follicular hyperplasia or follicular lymphoma.
Follicular lymphoma
The neoplastic cells of multiple myelome active the ________ receptor.
RANK receptor on osteoclasts
How is definitive diagnosis of mon made?
By serologic testing for the EBV viral capsid antigen
Characteristics of blasts
Large
Immature
punched out nucleoli
What sites are infected by EBV?
Oropharynx
Liver
B cells
Overexpression of cyclin D1 promotes _____ transition in the cell cycle, facilitating neoplastic proliferation.
G1/S
Gastric MALToma may regress with treatment of __________.
H.Pylori
Positive test for tartate-resistant acid phosphatase
Hairy cell leukemia
Subtypes of hodgkin lymphoma
- Nodular sclerosis
- Lymphocyte-rich
- Mixed cellularity
- Lymphocyte-depleted
Cytogenetic malfomation in Burkitt’s lymphoma
t(8,14), resulting in the translocation of c-myc on chromosome 8 to IgH on chromosome 14
Rouleaux formation of RBCs on blood smear
Multiple Myeloma
*Increased serum protein decreases charge betCween RBCs
All of the myeloproliferative disorders are associated with a JAK2 kinase mutation, except _________.
CML
Acute presentation of leukemia
Anemia (fatigue)
Thrombocytopenia (bleeding)
Neutropenia (infection)
In my cosis fungoides, aggregates of neoplastic cells in the epidermis are called _________.
Pautrier microabscesses
Clinical features of multiple myeloma
- Increased risk of infection
- Hypercalcemia
- Lytic lesions
- M Spike- Elevated serum protein
- Rouleaux formation
- Primary AL amyloidosis
- Proteinuria
Types of Acute Leukemia
Acute lymphoblastic leukemia
Acute myelogenous leukemia
Complications of most myeloproliferative disorders
- Increased risk for hyperuricemia and goat due to high turnover of cells
- Progression to marrow fibrosis or transformation to acute leukemia
Acute leukemia is the neoplastic proliferation of _________.
Blasts
*Accumulation of >20% blasts in the bone marrow
Types of Chronic Leukemia
- Chronic lymphocytic leukemia
- Hairy cell leukemia
- Adult cell leukemia/lymphoma
- Mycosis Fungoides
*Chronic leukemia is the neoplastic proliferation of mature circulating lymphocytes; characterized by a high WBC count
Which type of hodgkin has the best prognosis of all types?
Lyphocyte rich
CD1a+
S-100
Langerhans cell histiocytosis
Causes of lymphopenia
- Immunodeficiency
- High cortisol stae
- Autoimmune destruction
- Whole body radiation
- Bone pain with hypercalcemia
- M spike
- Anemia
- Bone lytic lesions
- Renal involvement
Multiple myelome
Where does T cell hyperplasia occur to cause splenomegaly in EBV infections?
T-cell hyperplasia in the periarterial lymphatic sheath (PALS), white pulp
What bacterial infection causes lymphocytic leukocytosis?
Bordetella pertussis
*Produce lymphocytosis-promoting factor, which blocks circulating lymphocytes from leaving the blood to enter the lymph node.
How do high cortisol states lead to neutrophilic leukocytosis?
Impairs leukocyte adhesion, leading to release of marginated pool of neutrophils
Clinical features of Adult T cell leukemia
- Rash
- Generalized lymphadenopathy with hepatosplenomegaly
- Lytic bone lesions with hypercalcemia
Treatment for Waldenstrom macroglobulinemia
Acute complications are treated with plasmapheresis, which removes IgM from the serum
Disruption of normal lymph node architecture. Follicular hyperplasia or follicular lymphoma.
Follicular lymphoma
Neoplastic proliferation of reed-sternberg cells
Hodgkin lymphoma
- blurry vission and headache
- Budd-Chiari Syndrome
- Flushed face
- Itching
Polycythemia vera
What types of NHL lead to proliferation of intermediate-sized B cells?
Burkitt’s lymphoma
How is polycythemia vera distinguised from reactive polycythemia?
- In PV, erythropoietin levels are decreased and SaO2 is normal
- In reactive polycythemia , SaO2 is low and EPO is increased
- In reactive polycythemia due to ectopic EPO production from renal cell carcinoma, EPO is high and SaO2 is normal.
Chronic myeloid leukemia is driven by t(9,22) which generates a BCR-ABL fusion protein with increased ________ activity.
Tyrosine kinase
Tingible body macrophages. Follicular hyperplasia or follicular lymphoma.
Follicular hyperplasia
*Cells undergo apoptosis in follicular hyperplasia. They do not in Follicular lymphoma
What cytogenic traits determine the prognosis of ALL?
- t (12,21)- good prognosis
- t (9,22)- poor prognosis
Viral infection will lead to what type of lymph node hyperplasia?
Paracortex
ALL has positive staining for _________. AML has positive staining for _________.
TdT; myeloperoxidase (MPO)
*TdT is found when staining the nucleus. MPO is found when staining the cytoplasm
Types of Acute myeloid leukemia
- Acute promyelocytic leukemia
- Acute monocytic leukemia
- Acute megakaryoblastic leukemia
What is the most definitive characteristics of lymphoblasts ?
TdT
*TdT absent in myeloid blasts and mature lymphoctes
What are common symptoms of hodgkin lymphoma? What do these symptoms present?
- Fevers, chills, weight loss, and night sweats
- may lead to fibrosis
- Reed- Sternburg cells secrete cytokines
What is the CD8 T cell response to EBV?
- Generalized lymphadenopathy
- Splenomegaly
- High WBC count with atypical lumphocytes in the blood
Myelofibrosis blood smear
Leukoerythoblastic smear
- Tear drop RBCs
- Nucleated RBCs
- Immature granulocytes
Cuases of lymphocytic leukocytosis
- Viral infections
- Bordetella pertussis
What types of NHL lead to proliferation of small B cells?
Follicular lymphoma
Mantle cell lymphoma
Marginal zone Lymphoma
Small lymphocytic lymphoma
Cytogenetic abnormalities seen in acute promelocytic leukemia
- t(15,17), which involves translocation of the retinioic acid receptor (RAR) on chromosome 17 to chromosome 15
- RAR disruption blocks maturatuon and promyelocytes accumulate
Which type of hodgkin is associated with abundant eosinophils?
mixed-cellularity
Causes of monocytosis
- Chronic inflammatory states
- Autoimmune or infectious
- Malignancy
Neoplastic proliferation of mature CD4 T cells that infiltrate the skin, producing localized skin rash, plaques, and nodules
Mycosis fungoides
Possible causes of reactive polycythemia
- Lung disease
- High altitude
- Ectopic EPO production from renal cell carcinoma
ALL presents in children with down syndrome ______ (before/after) age of five. AML presents in children with down syndrom ____ (before/after) age of five.
After; before
Treatment for neutropenia
GM-CSF or G-CSF
*Boost granulocyte production
Cytogenic marker for follicular lymphoma
t(14,18)
*BCL2 on chromosome 18 translocates to the Ig heavy chain locus on chromosome 14
High serum _______ may be present in multiple myeloma.
IL-6
*Stimulates cell growth and immunoglobulin production
What is the primary cause of symptoms seen in polycythemia vera?
Hyperviscocity of blood
Monoclonality. Follicular hyperplasia or follicular lymphoma.
Follicular lymphoma
Presentation of myelodysplastic syndromes
- Cytopenias
- Hypercellular bone marrow
- Abnormal maturation of cells
- Increased blast (<20%)
*Cells don’t get out into blood
Crystal aggregates of MPO may be seen as _______.
Auer rods
The african form of burkitt’s lymphoma involves the __________. The sporadic form involves the _________.
Jaw; abdomen
Cytogenetic problem in mantle cell lymphoma
t(11,14) in which Cyclin D1 gene on chromosome 11 translocates with IgH on chromosome 14
Complication of follicular lymphoma
Progression to diffuse large B-cell lymphoma
Chronic lymphocytic leukemia is the neoplastic proliferation of _________ cells that express CD5 and CD20.
Naive B Cells
How is CML distinguised from a leukemoid reaction?
- Negative leukocyte alkaline phosphaase stain
- Increased basophils
- t(9,22)
- Blasts infiltrate gums
- Lack MPO
Acute monocytic leukemia
_________ is used for screening for mono.
Monospot test
*Detects IgM antibodies that cross-react with horse or shep red blood cells (heterophile antibodies)
list examples of chronic inflammatory states associated with marginal zone lymphoma
- Hashimoto thyroiditis
- Sjogren syndrome
- H. pylori gastritis
Rheumatoid arthritis and early stages of HIV infection will lead to what type of lymph node hyperplasia?
Follicular
Complications of chronic lymphocytic leukemia
- Hypogammaglobulinemia
- Autoimmune hemolytic anemia
- Transformation to diffuse large B-cell lymphoma (Richter transformation)
Immature neutrophilic cells are characterized by decreased ______.
CD16 (Fc receptors)
*Results in decreased opsonization
Most common primary malignancy of bone
Multiple myeloma
- Co expression of CD5 and CD20
- Increased lymphocytes
- Smudge cells
Chromic lymphocytic leukemia
Causes of infectious mononucleosis
EBV
CMV *less common
- t(15, 17)
- Increased risk for DIC
Acute promyelocytic leukemia
Causes of neutrophilic leukocytosis
- Bacterial infection or tissue necrosis
- High cortisol state
*Induce release of marginated pool (both) and bone marrow neutrophils, including immature forms (infection/necrosis)
Neoplastic proliferation of mature myeloid cells, especially RBCs
Polycythemia vera
In chronic lymphocytic leukemia, involvement of lymph nodes leads to generalized lymphadenopathy and is called __________.
Small cell lymphoma
In hairy cell leukemia, where are hair cells trapped in the spleen?
Red pulp
Clinical features of Hairy Cell Leukemia
- Splenomegaly
- “Dry tap” on bone marrow aspiration
*Lymphadenopathy is usually absent
Clinical feautures of Waldenstrom macroglobulinemia
- Generalized lymphadenopathy
- Increased serum protein with M spike (IgM)
- Visual and neurologic deficits
- Bleeding
B-ALL is characteristized by lymphoblasts that express what markers?
CD10, CD19, CD20
___________ is the earliest change to emerge after whole body radiation.
Lymphopenia
Complications of mono
- Increased risk for splenic rupture
- Rash if exposed to ampicillin
- Recurrence (due to dormancy) and B cell lymphoma (in immunodeficiency)
First line of treatment for CML
Imatinib, which blocks tyrosine kinase activity
How does T-ALL present in teenagers?
As a mediastinal thymic mass called a lymphoblastic lymphoma
Which type of hodgkin is the most aggressive?
Lymphocyte-depleted
*Usually seen in the elderly and HIV-positive people
Draining of tissues with cancer will lead to what type of lymph node hyperplasia?
Sinus histiocytes (medulla)
ALL has an excellent response to chemotherapy but requires prophalaxis to _______ and ____.
Scrotum; CSF
Treatment of polycythemia vera
Phlebotomy
Hydroxyurea (second line)
Clinical features of myelofibrosis
- Splenomegaly due to extramedullary hematopoiesis
- Leukoerythroblastic smear
- Increased risk of infection, thrombosis, and bleeding
Hematopoiesis occurs via a stepwise maturation of _______ hematopoietic stem cells.
CD 34+
Causes of painful lymphadenopathy
Lymph nodes draining a region of acute infection
Adult T-cell leukemia is the neoplastic proliferation of ___________ .
Mature CD4 T cells
Causes of neutropenia
- Drug toxicity
- Damage to stem cells results in decreased production of WBCs, especially neutrophils
- Severe infection
Essential thrombocythemia is assocated with ________ mutation.
JAK2 kinase
Why does a high cortisol state lead to lymphopenia?
Induces apoptosis of lymphocytes
In CML, _________ suggests progression to accelerated phase of disease.
Splenomegaly
*Transformation to acute leukemia usually follows shortly thereafter
Primary effusion lymphoma associated with which virus
HHV8
Which form of MALToma is resistant to antibiotics?
t(11,18)
Which lymphoma is associated with a BRAF mutation?
Hairy cell leukemia
Fried egg appearance in bone marrow
Hairy cell leukemia
High Ki67
Diffuse large b cell lymphoma
CD38
Plasma cells
Stains for amyloid
Congo red stain
Apple green birefringence
CD20+, CD30-, CD15-
Nodular lympocyte predominant hodgkin
Conditions associated with a thymoma
Epithelial neoplasm of thymus
Myastenia gravis
Pure red cell aplasia