WBC disorders Flashcards
Acute Promyelocytic Leukemia (APL) is characterized by
t(15;17) of Retinoic Acid Receptor (RAR)
RAR disruption blocks maturation and promyelocytes accumulate
Adult T-cell leukemia/lymphoma clinical features
- Rash
- Generalized lymphadenopathy with hepatosplenomegaly
- Lytic bone lesions with hypercalcemia
ALL is
neoplastic accumulation of lymphobasts in the BM
ALL most commonly arises in
Children with Down Syndrome
APL can increase the risk for
DIC
B-ALL characterized by
- TdT
- CD10
- CD19
- CD20
Basophilia seen in
Chronic Myeloid Leukemia
Blasts are
Large immature cells often with punched out nucleoli
Causes of Lymphopenia
- Immunodeficiency
- High cortisol state induces apoptosis of lymphocytes
- Autoimmune destruction
- Whole body radiation
CD8 T-cell response to Infections mononucleosis leads to
- Generalized lymphadenopathy due to T-cell hyperplasia
- Splenomegaly due to T-cell hyperplasia in the PALS
- High WBC count with atypical lymphocytes
Clinical features of Hairy Cell Leukemia
- Splenomegaly: accumulates in red pulp
- Dry tap on BM aspiration due to marrow fibrosis
CLL complications include
- Hypogammaglobulinemia: most common cause of death
- IgM autoimmune hemolytic anemia
- May transform to diffuse large B-cell lymphoma (Richter transformation)
CLL naive B cells co-express
CD5 and CD20
CML is
Neoplastic proliferation of mature myeloid cells, especially granulocytes and their precursors
CML translocation is
t(9;22) philadelphia chromosome generates BCR-ABL fusion that increases tyrosine kinase activity
Complications of Myeloproliferative disorders (MPD) are
- Increased risk of hyperuricemia and gout due to high turnover of cells
- Progression to Marrow fibrosis or transformation to acute leukemia
EBV primarily infects
- Oropharynx: pharyngitis
- Liver: hepatits and hepatomegaly with elevated liver enzymes
- B cells
Eosinophilic granuloma is
Benign proliferation of Langerhans cell in bone
No skin involvement
Essential thrombocytothemia is
Neoplastic proliferation of mature myeloid cells especially plateletsb
ET is associated with what mutation
JAK2 kinase
First line treatment of CML is
Imantinib
Follicular hyperplasia of lymph node seen in
RA
Early signs of HIV
Follicular lymphoma can progress to
diffuse large B-cell lymphoma
Follicular lymphoma is
Neoplastic proliferation of small B cells (CD20+) that form follicle-like nodules
Follicular lymphoma treatment
Only for patients who are symptomatic
Chemotherapy or rituximab
Hairy cell leukemia cells are positive for
TRAP
Hairy cell leukemia is
Neoplastic proliferation of mature B cells characterized by Hairy cytoplasmic processes
Hand-Schüller-Christian disease
Malignant Langerhans cells
Hand-Schüller-Christian disease presentation
- Scalp rash
- Lytic skull defects
- DI
- Exophthalmos in children
Immature cells are characterized by
Decreased Fc receptors (CD16)
In what disorder do blasts infertrate gums
Acute monocytic leukemia
Infectious mononucleosis is caused by
EBV infection that results in Lymphocytic lekocytosis
Lacunar cells are seen in what type of Hodgkins lymphoma
Nodular sclerosis
Letterer-Siwe disease is
Malignant proliferation of langerhans cells
Skin rash with cystic skeletal defects
Leukocytosis is
Increase WBC
Leukopenia is
Decreased WBC
Lymphoblasts in ALL are characterized by
Positive nuclear staining for TdT
Lymphopenia refers to
A decreased number of circulating lymphocytes
M spike in Multiple myeloma due to
Monoclonal IgG or IgA
Mantel cell lymphoma translocation
t(11;14) causing an overexpression of cylcin D1 promotes G1/S transition
Marginal zone lymphoma associated with
- Hashimoto
- Sjögren
- H pylori gastritis
Mediastinal (thymic) mass in teenagers
T-ALL
Multiple myeloma is
Malignant proliferation of plasma cells in the BM
High serum IL-6
Mycosis fungoides is
neoplastic proliferation of mature CD4 T cells that infiltrate the skin producing localized skin rash, plaques, and nodules
Myeloblasts are characterized by
Positive cytoplasmic staining for MPO
MPO crystal aggregates may be seen as Auer Rods
Neutropenia is
Decreased number of circulating PMN
Non-Hodgkins lymphoma include
- Small B cell
- Follicular
- Mantel cell
- Marginal zone
- Small lymphocytic
- Burkitt lymphoma
- Diffuse large B-cell lymphoma
Pautrier microabscesses are
aggregates of neoplastic cells in the epidermis seen in Mycosis fungoides
Polycythemia vera is associated with what mutaiton
JAK2 kinase mutation
PV clinical symptoms
- Blurry vision and headache
- Increase risk of Venous thrombosis
- Flushed face due to congestion
- Itching, especailly after bathing
Reed-Sterberg cells are
- Malignant cells seen in Hodgkin lymphoma
- Large B cels with multilobbed nuclei with prominent nucleoli (‘owl-eyed nuclei’)
- Positive for CD15 and CD3-
Rouleaux formation of RBC is
Increases serum protein decreases charge between RBCs attaching them together seen in Multiple Myeloma
Sezary cells are
Lymphocytes with cerebriform nuclei seen on blood smear of Mycosis Fungoides
T-ALL characterized by
TdT
CD2 to CD8
Waldenström Macroglobulinemia is
B-cell lymphoma with monoclonal IgM production
What causes Neutropenia
- Drug toxicity damages stem cells
- Severe infection from G(-) causing an incresed movement of PMN to tissue
What is follicular lymphoma translocation
t(14;18) leading to overexpression of BCL2
What is seen on CLL blood smear
Increased lympohcytes and smudge cells
What is the treatment of APL
All-trans-retinoic acid
What is used to treat Hairy cell leukemia
- 2-CDA (cladribine)
- Adenosine deaminase inhibitor
What translocation of B-ALL has bad prognosis
t(9;22)
What translocation of B-ALL has good prognosis
t(12;21)