clean up Flashcards
1
Q
- Proliferation of immature BM cells, with > 20% blasts (PB or BM)
- Associated with Down syndrome after 5 y/o
A
• Acute lymphocytic leukemia (ALL)
2
Q
- Symptoms: aggressive, pancytopenia due to BM replacement
- Good: hyperdiploidy, t(12,21), 2 – 10 y/o
- Poor: hypodiploidy, t(9,22), 11q23 (MLL), CNS involvement, WBC > 100,000/µL
- First-line tx for Ph+ adults is dasatinib
- Excellent response to chemo, but scrotum and CNS require “prophylaxis”
A
• B lymphoblastic leukemia (B-ALL)
3
Q
• Worse prognosis than B-ALL
A
• T lymphoblastic lymphoma (T-ALL)
4
Q
- Symptoms: indolent, hypogammaglobulinemia, sometimes autoimmune hemolysis and thrombocytopenia
- Immunophenotype: CD5+, CD19+, CD20+, CD23+
- B cells with chromatin that looks like soccer balls or gingersnaps
- Presence of smudge cells on PBS
- Risk of Richter transformation to large B cell lymphoma
- Good: del13q, low Rai stage, mutated IgHV
- Intermediate: trisomy 12
- Poor: del11q, del17p, high Rai stage, unmutated IgHV, CD38+, ZAP70+
- Incurable, 5-yr survival is 75%
A
• Chronic lymphocytic leukemia (CLL)
5
Q
- Symptoms: indolent, pancytopenia, splenomegaly (red pulp, which is unique), BM fibrosis (dry tap), absence of lymphadenopathy
- TRAP expression (=osteoclastic activity)
- Immunophenotype: CD19+, CD20+, CD11c+, CD22bright
- Cells with hairy projections and reniform nuclei
- Risk of Richter transformation to large B cell lymphoma
A
• Hairy cell leukemia (HCL)
6
Q
- Proliferation of immature BM cells, with > 20% blasts (PB or BM)
- Natural course is fatal within weeks to months
- Comprises 80-90% of acute leukemias
- NSE+ (monoblasts only)
A
• Acute myelogenous leukemia (AML)
7
Q
- De novo AML; no antecedent MDS
* Generally favorable prognosis
A
• AML with recurrent cytogenetic abnormalities
8
Q
- DIC is common, contributes to early morbidity and mortality
- Atra Quickly corrects coagulopathy
- Morphology: atypical promyelocytes, generally hypergranular, reniform nuclei, multiple Auer rods ( can trigger coagulation DIC)
A
• AML with t(15;17) – acute promyelocytic leukemia (APL)
9
Q
- Hypergranular form
- Hypergranular cells
- Leukopenia
- Hypogranular form
- Hypogranular (microgranular) cells
- Leukocytosis
A
• AML with t(15;17) – acute promyelocytic leukemia (APL)
10
Q
• Morphology: dysplastic BM eosinophils, myelomonocytic immunophenotype
A
• AML with inv(16)
11
Q
• Common AML among infants
A
• AML with 11q13 (MLL) rearrangement
12
Q
- Dysplasia in > 50% of cells in 2 or more lineages
- Typically an antecedent MDS
- Karyotypic abnormalities (e.g. del7q, -7, del5q, -5)
- Occurs in older people
- Poor prognosis
A
• AML with myelodysplastic-related changes
13
Q
- Cytogenetic abnormalities in chromosome 5 or 7
- Multilineage dysplasia (resembles AML with myelodysplastic-related changes)
- Very poor prognosis
A
• Alkylating agent related AML/MDS
14
Q
- 11q23 (MLL) translocations are the most common
- Monocytic or myelomonocytic (resembles de novo AML with 11q13 rearrangement)
- Poor prognosis
A
• Topoisomerase II inhibitor related AML/MDS
15
Q
• Monocytes characteristically invade the gums
A
- AML not otherwise specified (AML NOS)
* Acute monocytic leukemia
16
Q
- Leukocyte alkaline phosphatase (LAP) negative
* Basophilia highly associated with CML (not seen in leukemoid reactions)
A
• Chronic myelogenous leukemia (CML)
17
Q
- Affects adults and is rare
- Immunophenotype: CD8+
- Lymphocytes with eosinophilic granules
- Symptoms: indolent, neutropenia, anemia, splenomegaly, may be associated with autoimmune disease
A
• Large granular lymphocytic leukemia
18
Q
• Lymphocytes with lobated nuclei (flower cells)
A
• Adult T cell leukemia/lymphoma (ATLL)
19
Q
• Post-germinal B cells with crippling Ig mutations no Ab secretion
A
• Hodgkin’s lymphoma
20
Q
- Most common subtype
* Fibrous bands with lacunar Reed-Sternberg cells
A
• Nodular sclerosis (NSHL)
21
Q
• Lack fibrous bands and nodules, more commonly caused by EBV, more commonly has BM involvement
A
• Mixed cellularity (MCHL)
22
Q
- Median age 35 y/o, mostly males, nodular lesions, no EBV-association
- Popcorn cells, no eosinophils or plasma cells
- Immunophenotype: CD20+, CD45+, CD15 negative, CD30 negative
- Can be treated more conservatively than classic Hodgkin’s lymphoma
- Good prognosis
A
• Lymphocyte rich (NLPHL)
23
Q
- Immunophenotype: CD10+, CD20+, CD5 negative
- Presents in late adulthood with generalized painless lymphadenopathy
- Tx symptomatic patients with low dose chemo, rituximab
- May progress to large B cell lymphoma
A
• Follicular cell lymphoma
24
Q
- Immunophenotype: CD5+, CD20+, CD23 negative
* Presents in late adulthood with generalized painless lymphadenopathy
A
• Mantle cell lymphoma
25
Immunophenotype: CD5 negative, CD10 negative
• MALToma
26
* Most common kind of non-Hodgkin’s lymphoma
* Presents in late adulthood as an enlarged LN or extranodal mass
* Aggressive
• Diffuse large B cell lymphoma (DLBCL)
27
* Caused by c-MYC translocations
* With IgH t(8;14)
* With kappa t(2;8)
* With lambda t(8;22)
* Immunophenotype: CD10+, CD19+, CD20+, Bcl2+, KI-67+
* Aggressive cancer that presents in as an extranodal mass in adolescents and adults
* Starry sky appearance on histology
• Burkitt’s lymphoma
28
* EBV-related
* Endemic to Africa
* Affects the jaw
• Endemic
29
• Affects the abdomen
• Sporadic
30
• EBV-related
• Immunosuppression-related
31
• Caused by ALK-NPM rearrangement t(2;5)
| Aggressive lymphoma of children and young adults, more common in males
• Anaplastic large cell lymphoma
32
• Lymphocytes with cerebriform nuclei and powdery chromatin
• Cutaneous T cell lymphoma
33
* BRAF mutations
| * CD1a+, MHC-II+, langerin+ (found in Birbeck granules, look like tennis rackets)
• Langerhans cell histiocytosis (LCH)
34
* Painful bone lesions
| * Peak incidence between 5 – 10 y/o
• Eosinophilic granuloma
35
* Rapid proliferation of Langerhans cells in multiple tissues
* Mostly seen in children
• Letterer-Siwe disease
36
* Very high ferritin (>10,000µg/L)
* Hypertriglyceridemia, hypofibrinogenemia, hemophagocytosis
* 1-yr survival with SCT is 92%; without treatment, 0%
• Hemophagocytic lymphohistiocytosis (HLH)
37
• Caused by mutations related to perforin or granzyme secretion
• Primary (familial) HLH
38
• Occurs after strong immunologic activation (e.g. EBV, leukemias, lymphomas, autoimmunity)
• Secondary (acquired) HLH
39
* Symptoms: malaise, SOB, headaches, night sweats, bleeding from gums or gut, hepatosplenomegaly, gout, venous thrombosis, Budd-Chiari syndrome, face may look red, itching especially after showers (due to increased mast cells)
* Tx is phlebotomy, aspirin, hydroxyurea, anagrelide
• Polycythemia vera
40
* Elevation of platelets with abnormal morphology (large, hypogranular)
* Occurs in people 50 – 70 y/o, also young females
* Tx is aspirin, hydroxyurea, anagrelide, alkylating agents, SCT
• Essential thrombocythemia
41
* Extramedullary hematopoiesis spleen, liver, LNs
* Leukoerythroblastic smear (BM shows trapped WBC and RBC)
* Immature RBC and WBC in the blood
* Anemia with misshapen RBC (teardrop cells)
* Occurs in people 50 – 70 y/o
* May develop into AML
* Tx is ruxolitinib (JAK2 inhibitor), hydroxyurea, SCT
• Primary myelofibrosis
42
* Caused by del5q, -5, del7q, +8
* Dysplastic HSCs with ineffective hematopoiesis
* Dysplastic neutrophils are hyolobated and hypogranular
* Dysplastic platelets are large and hypogranular
* Cytopenias (uni, bi, or pan)
* Myeloblasts are increased but are still
• Myelodysplastic syndrome (MDS)
43
* Symptoms: anemia, bacterial infections, pathologic bone fractures, bone pain, proteinuria, proteinemia, hypercalcemia, renal failure
* Tx with chemo, thalidomide derivatives, proteasome inhibitors (bortezomib, carfilzomib), corticosteroids, autologous SCT, radiation for local lesions
• Multiple myeloma
44
* Symptoms: hyperviscosity syndrome, visual impairment, Raynaud’s syndrome, autologous IgM (rheumatoid factor, cold agglutinins)
* Tx is plasmaphoresis, corticosteroids, alkylating agents, nucleoside analogs, rituximab, bortezomib, thalidomide, lenalidomide
• Waldenström's macroglobulinemia (lymphoplasmacytic lymphoma)
45
• Symptoms: typically asymptomatic, peripheral neuropathy, bone fractures, primary amyloidosis
• Monoclonal gammopathy of undetermined significance (MGUS)
46
* Two-month incubation period before symptoms arise
| * Oncogenesis may be direct (transformation) or indirect (chronic inflammation-inducing hit-and-run bystander)
• Infectious mononucleosis
