FA + MTB 3 Flashcards
T cell ALL can present as
Mediastinal mass (presenting as SVC-like syndrome)
Acute lymphoblastic leukemia/lymphoma (ALL)-better prognosis if
t(12;21)
Acute lymphoblastic leukemia/lymphoma (ALL) may spread to
CNS and testes
Acute lymphoblastic leukemia/lymphoma (ALL) markers
- TdT+ (pre-T and pre-B cells)
2. CD10+ (pre-B cells only)
CLL markers
CD20 and CD5 and CD23 B cell neoplasm
Smudge cells in the peripheral smear leukemia
CLL
Richter transformation
CLL/SLL transformation into aggressive large B cell lymphoma
Hairy cell leukemia
Age and type of cells
Adults males
Mature B cells (with hair-like cells projections) –> fuzzy appearing on LM
Hair cell leukemia cells morphology/presentation
Cells with filamentous, hair-like cells projections (fuzzy appearing on LM)
presents with massive splenomegaly
peripheral lymphadenopathy is very uncommon
Hair cell leukemia - aspiration?
dry because of Marrow fibrosis
Hair cell leukemia cells detection
- Stains TRAP (Tatrate-resistant acid phosphate)
2. Flow cytometry (largely replaced TRAP)
Hair cell leukemia treatment
- Cladribine
2. Pentostatin
Auer robs
Perioxidase + cytoplasmic inclusions seen mostly in M3 AML
AML risk factors
- Prior exposure to alkylating chemotherapy
- Radiation
- Myeloroliferative disorders
- Myelodysplastic
- Down syndrome
AML common presentation
DIC
AML M3 treatment
If t(15;17).. M3 subtype respond to all trans retinoic acid (vit A), inducing differentiation of meyloblasts
Philadelphia ch
t(9;22)
BCR-ABL
CML cells
Mature and Maturing granoulocytes
Neutrophils
Metamyelocytes
Basophils
Blast crisis
CML transform to AML or ALL
CML vs leukemoid reaction
CML has very low LAP as a result of low activity in malignant neutrophils (vs benign neutrophilia)
t8;14
Burkitt lymphoma (c-myc)
t9;22
Philadelphia (BCR-ABL)
- CML
- rarely ALL
t11;14
Mantle (cyclin d1)
t14;18
Follicular lymphoma (BCL-2)
t12;21
Better prognosis ALL
t15;17
M3 AML subtype respond to vit A
Langerhans cell histiocytosis present in a child as
- Lytic bone lesions
- Skin rash
- Recurrent otitis with a mass involving the mastoid
Langerhans cell histiocytosis - T cells activation
Cells are functionally immature and do not effectively stimulate primary T cells via antigen presentation
Langerhans cell histiocytosis markers
S-100 (mesodermal origin)
CD1a
Histological feature of Langerhans cell histiocytosis
Birdeck granules (tennis racket or rob shape on EM) are characteristic
Polycythemia vera definition/symptoms
A form of 1ry polycythemia –> disorder oh high Hct
- Intense itching after hot shower (due to increased basophils)
- Erythromelalgia (rare, severe, burning pain and red blue discoloration) due to blood clots in vessel
Polycythemia vera respond to
phlebotomy, hydroxyuream ruxolitinib (JAK1/2 inhibitor)
types of polyceythemia
- relative
- Appropriate absolute
- inappropriate absolute
- Polycythemia vera
types of polyceythemia and O2 saturation
- relative: normal
- Appropriate absolute: decreased
- inappropriate absolute: normal
- Polycythemia vera: normal
Relative polycythemia - associations
- dehydration
2. burns
Appropriate absolute polycythemia - associations
- lung disease
- congenital heart disease
- high altitude
Inappropriate absolute polycythemia - associations
- malignancy: renal, hepatocellular etc
- hydronephrosis
(ECTOPIC EPO secretions)
Essential thrombocytosis definition / symptoms
massive proliferation of megakaryocytes and platelets
symptoms: 1. Bleeding 2. Thrombosis
3. Erythromelalgia may occur
Myelofibrosis - definition
Obliteration of bone marrow due to increased fibroblast activity in response to proliferation of monoclonal cell lines
(pathoma: neoplastic prolif of mature myeloid cells, esp megak –> PDGF –> marrow fibrosis)
Chronic myeloproliferative disorders - JAK2
Polycythemia vera: +
Essential thromb: + (30-50%)
Myelofibrosis: + (30-50%)
CML: -
Chronic myeloproliferative disorders - PHILDELFIA
CML
Polycythemia vera - cells
increased RBC, WBC, platelets
Essential thromb - cells
increased platelets
myelofibrosis - cells
decreased RBCs
variable WBCs and platelets
CML
decreased RBCs
increased WBCs and platelets
lung cancer - who should be treated with surgery
- the size is not the most important factor
- if the lesions is large but is surrounded by normal lung and there is enough remaining lung function post resection, the surgery is still possible
lung cancer - the surgery is not possible if
- bilateral disease or lymph nodes involved on opposite size
- malignant pleural effusion
- heart, carina, aorta, or cena cava is involved
lung cancer screen
annually with low-dose chest cancer in those with:
adults 55-80 who with 30 pack year smoking history
ovarian cancer - most accurate test
ovarian cancer - initial test
most accurate: biopsy
initial: U/S
ovarian cancer - treatment
the only cancer in which removing large amounts of locally metastatic disease will benefit the patient. Remove all visible tumor and pelvic organs and give chemotherapy
Azathioprine, 6-mercaptopurine, 6-thioguanine side effects
- Myelosuppresion 2. GI. 3. Liver
Cladribine (2-CDA) clinical use / toxicity
Hairy cell leukemia
- Myelosupression
- Nephrotoxicity
- Neurotoxicity
Cytarabine (arabinofuranosyl cytidine) clinical use / toxicity
AML LYMPHOMAS 1. Leukopenia 2. Thrombocytopenia 3. Megaloblastic anemia
Methotrexate (MTX) toxicity
- Myelosupression (REVERSIBLE WITH LEUCOVORIN)
- Hepatotoxicity
- Mucositis (mount ulcers)
- Pulmonary fibrosis
5-fluorouracil toxicity
Myelosupression (NOT REVERSIBLE WITH LEUCOVORIN)
Antitumor antibiotics
- Bleomycin
- Dactinomycin (actinomycin D)
- Doxorubicin, daunorubicin
Bleomycin side effects
- Pulmonary fibrosis
- MINIMAL myelosupression
- Skin hyperpigmentation
Dactinomycin (actinomycin D) side effects
Myelosuppression
Doxorubicin,
Daunorubicin side effects
Toxic to tissues following extravasation
- Dilated cardiomyopathy
- Alopecia
- Myelosuppression
How to prevent dilated cardiomyopathy with doxorubicin or daunorubicin administration
Dexrazoxane, an iron chelatin agent
Alkylating agents
Busulfan
Cyclophosphamide, isosfamide
Nitrosoureas (carmustine, iomustine, semustine, streptozocin)
Busulfan side effects
- Severe myelosuppression (almost always)
- Pulmonary fibrosis
- Hyperpigmentation
Nitrosoureas toxicity
Cns: 1. Dizziness 2. Ataxia 3. Convulsion
Cyclophosphamide, ifosfamide toxicity
- Myelosuppresion
2. Hemorrhagic cystitis
Partially Prevent hemorrhagic cystitis in Cyclophosphamide, ifosfamide by:
Mesna (thiol group of mesna binds toxic metabolitis)
Microtubule inhibitors
- Paclitaxel, and others taxols
- Vincristine
- Vinblastine
Paclitaxel toxicity
- Myelosuppression
- Alopecia
- Hypersensitivity
- Neuropathy
Vinblastin toxicity
Myelossupression
Vincristine toxicity
- Neurotoxicity: (areflexia, peripheral neuritis)
2. Paralytic ileus
Hydroxyurea clinical use
- Melanoma
- CML
- Sickle cell anemia (increases HbF)
Hydroxyurea toxicity
- SEVERE myelosuppression
2. GI upset
Imatinib clinical use
- CML (BCR-ABL)
2. GI stromal tumors (c kit)
Imatinib toxicity
Fluid retention
Rituximab toxicity
Increased risk of progressive multifocal leukoencephalopathy
Cisplatin carboplatin toxicity
- Nephrotoxicity
- Ototoxicity
- peripheral neuropathy
How to prevent cisplatin or carboplatin associated nephrotoxicity
Amifostine (free radical scavenger) chloride (saline) diuresis
Etoposide, tenoposide - mechanism of action
Inhibits topoisomerase 2–> dna degradation
Etoposide tenoposide toxicity
- Myelosuppression
- Gi upset
- Alopecia
Irinotecan, topotecan mechanism of action
Inhibit topoisomerase 1 and prevent DNA unwinding and replication
Irinotecan, topotecan toxicity
- Severe myelosuppression
2. Diarrhea
Trastuzumab (herceptin) toxicity / clinical use
Cardiotoxicity
Her2 + breast cancer and gastric cancer
SERMs (tamoxifen, raloxifene) clinical
Tamoxifen: breast cancer treatment
Raloxifene: prevent osteoporosis
Both: breast cancer prevention