Flashcards in Robbins CH 13 WBCs Deck (107):
Syndrome caused by overwhelming acute infection. Petechial hemorrhages occur and there is presence of granulocytopenia?
Systemic inflammatory response syndrome
What are the causes of neutrophilic leukocytosis?
Chronic infections and ongoing inflammatory conditions--lung abscesses
What can prolonged glucocorticoid use cause?
Increased release of marrow storage pool cells and diminish extravasation of neutrophils into tissues
What type of leukemia is associated with basophilia?
Chronic myelogenous leukemia
Eosinophilia is a feature of what conditions?
Tissue parasitic infections
Cytopenia seen with acute infections and inflammatory conditions?
What are toxic granulations?
Coarse and dark primary granules seen with neutrophils that indicate an overwhelming inflammatory condition
What are Dohle bodies?
Patches of dilated ER of neutrophils indicating an overwhelming inflammatory condition
Patient presents with leukocytosis and immature myeloid cells in the peripheral blood. A high leukocyte alkaline phosphatase (LAP) score is present. What is the reaction occurring in this patient?
If abnormal maturation of myeloid cells in marrow and low LAP--> CML
What is seen on a blood smear of a patient with infectious mononucleosis?
Large atypical lymphocytes with abundant cytoplasm
Large nucleus with fine chromatin
What is the cause of infectious mononucleosis, be specific?
EBV genes--cause proliferation and activation of multiple cones of B cells--Polyclonal B-cell expansion
What cells are not increased with infectious mononucleosis?
What is present in lymph nodes draining cancer?
Reactive pattern, with dilated sinusoid that have endothelial hypertrophy and filled with histiocytes
What are seen on biopsy of lymph nodes caused by a reactive condition (painful)?
Large, variably sized, germinal centers containing numerous mitotic figures
Numerous parafollicular and sinusoidal neutrophils
What is CD3?
What is CD19 and 20?
What is CD68?
Macrophage (histiocyte) marker
A 5 year old presents with enlarged axillary lymph nodes and a raised erythematous nodule over abrasions. Histology is performed and stellate, necrotizing granulomas are seen. The mother states that she thinks the child was bitten by something. What is the most likely cause of this patients symptoms?
Cat-scratch disease--Bartonella henselae
A 7 year old patient present with a mediastinal mass that expresses CD2 and CD7. The patient has a point mutation in NOTCH1 gene. What do the markers represent and what is the dx?
Pre-T cell types
Acute lymphoblastic leukemia/lymphoma (ALL)
What does a + TdT marker indicate?
Pre-T and Pre-B cells
56 year old pt presents with non-tender axillary and cervical lymphadenopathy. blood smear shows monotonous population of small, round, mature-looking lymphocytes. Flow cytometry is + for CD19 and 5. What is the Dx?
Chronic lymphocytic leukemia (CLL)
What translocation is present with Burkitt lymphoma?
What translocation is present with chronic myeloid leukemia (CML)?
What translocation is present with follicular lymphomas?
What are features that support an acute leukemia?
Presence of blasts in the peripheral blood and bone marrow
What subtype of acute lymphoblastic leukemia has a good response to chemo, what age group are these patients in and what translocation is present?
Early pre-B cell type--hyperdiploidy
Patients btwn ages 2 and 10
Chromosomal trisomy and t(12;21)
What subtype of acute lymphoblastic leukemia has a poor response to chemo/poor prognosis, what age groups, what translocation and what prognostic factors?
What leukemia appears in young to middle-aged adults with peroxidase-postive myeloblasts?
Acute myelogenous leukemia
What cells increase in circulation in chronic lymphocytic leukemia?
Many small circulating mature B lymphocytes with scant cytoplasm
What do the cells express in chronic lymphocytic leukemia?
Is chronic lymphocytic leukemia seen in younger or older ind?
A condition in which platelet destruction is caused by an antibody-mediated process. Lab values show all normal cells except platelet count is reduced?
Idiopathic thrombocytopenic purpura
What type of pattern is seen with follicular lymphoma?
nodular or follicular pattern
What translocation is seen with follicular lymphoma?
What does the 14;18 translocation cause in follicular lymphoma?
Overexertion of the BCL2 gene by juxtaposing it with the IgH locus--cells resistant to apoptosis
Is there bone marrow involvement with acute lymphadenitis?
What type of cell are involved in a mantle cell lymphoma?
What translocation is seen with mantle cell lymphoma?
What is caused by the 11;14 translocation seen with mantle cell lymphoma?
Overexertion of the cyclin D1 gene-causing G1-S phase transition
What B cell lymphoma is commonly seen in HIV positive patients? How does this occur?
Diffuse large-cell lymphoma
Immunosuppression allows unregulated proliferation and neoplastic transformation of EBV-infected B-cells
What are the 2 different Burkitt lymphomas and which one is positive for EBV?
Sporadic-- seen in young children EBV-negative
Endemic-- seen in african children EBV positive
B-cell lymphoma that typically appears in the maxilla or mandible of the jaw?
Endemic Burkitt lymphoma seen in african children which is EBV +
What translocation is present in Burkitt lymphoma?
8;14--tranlocation of the the MYC gene on ch 8
57 year old patient present with a waldeyer's tonsillar ring. Biopsy is performed and the cells are CD19 and 10 positive. A BCL6 mutation is present. The disease responds well to chemo, but is rapidly fatal if it goes untreated. What is the dx?
Diffuse large B-cell lymphoma
1/3 of the cases have BCL6 mutation
T-cell CD3 ABSENT
Monocytic CD15 ABSENT
T-cell neoplasm that occur typically in the mediastinum of children?
Characterized by Reed-Sternberg cells?
A B-cell neoplasm that manifest with widespread lymphadenopathy, liver and spleen enlargement, and lymphocytosis?
Small lymphocytic lymphoma
Patient presents with bone lysis and pain. A skull X-ray shows punched-out lytic lesions--caused by plasma cells. Urinalysis shows Bence Jones proteinuria. Hypercalcemia and high serum IL-6 is found. What is the dx?
What causes the hypercalcemia seen with multiple myeloma?
cell production of MIP1-a which up-regulates RANKL production and increases osteoclast activity--punched out lytic lesions
Why are patient with multiple myeloma more susceptible to infection with encapsulated bacteria?
Decreased IgG production
Hyperviscosity syndrome involving visual disturbances, HA, Raynaud phenomena, hepatosplenomegaly, lymphadenopathy and very high protein (IgM)?
Lymphoplasmactic lymphoma--waldenstrom macroglobulinemia
Lymphoplasmactic lymphoma--waldenstrom macroglobulinemia
Bone marrow infiltrated with plasmacytoid lymphocytes that have stored immunoglobulins in their cytoplasm
What spike is seen with Lymphoplasmactic lymphoma--waldenstrom macroglobulinemia?
IgM spike caused by neoplastic B-cell differentiation to IgM-producing cells
Disease characterized by the presence of an M protein spike in the absence of any associated disease of B cells. the dx is made when M spike is small and the patient has NO Bence jones proteinuria.
Monoclonal gammopathy of uncertain significance (MGUS)
disease caused by 11;14 translocation causing the activation of cyclin D1 gene and also does not respond well to chemo.
Mantle cell lymphoma
A lymphoma that arises in middle-aged adults at sites of autoimmune or infectious stimulation. Most common sites are thyroid, salivary glands, stomach. Disease can regress with antibiotic therapy, but may also transform into diffuse large B-cell lymphoma...
Marginal zone lymphoma--MALT lymphoma or MALToma
What CDs are + with hairy cell leukemia?
What are the 2 defining characteristics of Hairy cell leukemia?
Presence of hairy projections from neoplastic leukocytes in the peripheral blood smear
Coexpression of B-cell (CD19 and 20) and monocyte (CD11c) markers
What test is used to ID the cells of hairy cell leukemia?
What causes the pancytopenia seen in hairy cell leukemia?
poor production of hematopoietic cells in the marrow and sequestration of the mature cells in the spleen
Where are auer rods seen?
in myeloblasts in acute myeloblastic leukemia
What characteristic gene rearrangement is seen with anaplastic large-cell lymphoma and what does this cause?
2p23 gene rearrangement--causes production of anaplastic lymphoma kinase (ALK) with tyrosine kinase activity
What is CD10 a marker for?
Malignancies of CD4+ and CD3+ Tcells that involves the skin and there is presence of lymphocytes with complex cerebriform nuclei?
Cutaneous T-cell lymphomas
What infection causes cloverleaf cells on blood smear?
Bartonella henselae infection that presents with lymphadenopathy with microscopic stellate necrosis?
What is helicobacter pylori associated with?
Associated with MALToma
What are Reed-Sternberg cells?
Elaborate cytokines that promote an accompanying reactive cellular proliferation--forming blue of neoplastic mass
CD15+ large cells with multiple nuclei or a single nucleus with multiple nuclear lobes
A biopsy of a Hodgkin lymphoma mass with have what cells?
A type of hodgkins lymphoma with bands of fibrosis, is common in adult women, and commonly involves the mediastina?
Nodular sclerosis HL
Type of hodgkin lymphoma that tends to affect older men, express CD15 AND CD30 (which is marker on Tcell/Bcells/monocytes?
Mixed cellularity HL
Type of hodgkin lymphoma that has an abundance of reed-sternberg cells and a paucity of lymphocytes. Most cases present with advanced disease and EBV is present in over 90% of the cases?
Lymphocyte depletion variant HL
BCL6 gene rearrangements?
typical of diffuse large B-cell lymphomas
Deletions of 5q...
Typical of myelodysplastic syndrome
Found in polycythemia vera and other myeloproliferative diseases
characteristic of HL
Histiocytes with birbeck granules...
Characteristic of the langerhans cell histiocytoses
Myeloblasts are characteristic of...
acute myelogenous leukemia
A disease involving infiltration of the marrow and the reduction of normal hematopoiesis--anemia and thrombocytopenia. Auer rods are present on smear. Nose bleeds and bleeding gums are seen and blasts are CD33+.
Acute myelogenous leukemia (AML)
What CD marker is seen with acute myelogenous leukemia (AML)?
What do Dohle bodies indicate?
Indicative of marked inflammation--bacterial sepsis caused by reactive changes in mature neutrophils
What leukemia can cause DIC by release of granules from promyelocytes?
Acute promyelocytic leukemia (APL)
What translocation is seen with acute promyelocytic leukemia (APL)?
What does the 15;17 translocation, seen with acute promyelocytic leukemia, cause?
Fusion of retinoic acid receptor gene on 17 with the promyelocytic leukemia gene on 15--blocking myeloid differentiation
What is the treatment for acute promyelocytic leukemia (APL)?
Retinoic acid (Vit A)
M2 variant of acute myelogenous leukemia
Peripheral blood count of leukocytes is not high, but leukemic blasts fill the marrow. Blasts are peroxidase negative and nonspecific esterase positive.
Acute monocytic leukemia
M5 leukemia is characterized by what?
high incidence of tissue infiltration and organomegly
very high WBC count
Presence of peroxidase-postive blasts filling the marrow. most often seen in ind. 15-39.
Acute myelogenous leukemia (AML)
Characterized by a cellular marrow in which there are maturation defects in multiple lineages. Dx confirmed by ringed sideroblasts, megaloblasts, abnormal megakaryocytes, and myeloblasts in the marrow.
Myelodyplasia develops either...
De novo or after chemo with alkylating agents
What is a marker of post therapy myelodyplasia?
chromosomal deletions--such as 5q
Ph chromosome 9;22
Tyrosine kinase activation
disease of pluripotent stem cells
Chronic myelogenous leukemia (CML)
Acute promyelocytic leukemias
Myeloproliferative disorder with thrombocytosis, increased megakaryocytes with no evidence of leukemia and presents with throbbing, burning pain in the extremities?
What causes the burning, throbbing pain seen with essential thrombocytosis?
platelet aggregates that occlude small arterioles
Results from an increased RBC mass with increased HCT and blood volume. Very low EPO levels and abnormal platelet function that predisposes pt to bleeding.
Polycythemia vera can blast out into...
Acute myelogenous leukemia and other patient develop chronic myelogenous leukemia
Teardrop RBCs are seen with what?
When marrow undergoes fibrosis in myelofibrosis with myeloid metaplasia
What disorder has Birbeck granules--tennis rackets?
Langerhans cell proliferations--langerhans cell histiocytosis
Hand-Schuller-Christain disease--diabetes insidious is seen in what disease?
Langerhans cell histiocytosis
A form of langerhans cell histiocytosis with birbeck granules..
Seen in anterior medistinal (thymic) masses in children with acute lymphoblastic leukemia/lymphoma?
Lymphoblasts that mark as T cells (CD3+)