intro to hematological malignancies Flashcards
What are hematopoietic malignancies
Clonal dx that are derived from a single cell in the marrow or peripheral lymphoid tissue that has undergone genetic alteration
percentage of hematologic cancers
7%
Risks for developing malignancy
Genetic inheritance
Environmental factors
Inherited factors causing malignancy
Downs syndrome Blooms syndrome Fanconis anemia Neurofibromatosis Klinefelters syndrome Familial tendency
Environmental factors that cause malignancy
Chemicals Drugs radiation infection viruses bacteria protozoa
How are oncogenes or protooncogenes activated
through translocation, mutation, or duplication
Oncogenes that suppress apoptosis
bcl-2
Tyrosine kinases are important for
intracellular signaling
Increasing tyrosine kinase activity found in the ff dx
CML-ABL JAK-2 in myeloproliferative dx FLT3 in AML Bruton kinase in CLL Lymphoproliferative disorders
Plasma cell dyscrasias differentiated into
Plasma cell myeloma
Plasmacytoma
Waldestrom macroglobulinemia
How de we take history in suspected hematological malignancy
Fever- persistent? intermittent? pel ebstein/ Jaundice Excess pruritus and sweating Chest pain and diffivulty in breathing Bleeding Recurrent infections
Physical examination of hematological malignancies
Anemia-signs and symptoms
CNS signs and symptoms which imply CNS involvement. i.e confusion, aggressive behavior
Thrombocytopenia i.e ecchymosis, purpura
Organomegaly i.e splenomegaly, hepatomegaly
Lymph adenopathy i.e lymphomas
Plasmacytomas- may be seen as myeloma or plasma cell dyscrasias
What to look for if suspecting leukemia
FBC and comment
Bone marrow aspirate for morphology, cytogenetics, etc
Trephine biopsy
Lumbar puncture; Csf needed for cytology
Chest radiography for mediastinal involvement
Abdominal ultrasonography fpr organomegaly
CT scan and MRI when needed
What is immunophenotyping
Helps to categorize cells by their surface antigens using antibodies labelled with different fluorochromes
B cell surface antigens include
CD19,20,22
T cell surface antigens include
CD-3,5,7
AML surface antigens include
CD 13,33,117
What is immunohistochemistry
The prescence and architecture of tumour cells can be identified and visualised under a microscope in stained tissue sections using monoclonal antibodies
Identification of chromosomal abnormalities is done by
Flourescent in situ hybridization - flourescent labelled genetic probes
Southern blot analysis -Extraction of DNA from leukemic cells
PCR - Detects translocations and gene rearrangements
Principles of management of malignancies
Clinical diagnosis- physical exam and history
Lab diagnosis
Treatment
Treatment of malignancies include
Specific - chemotherapy Supportive - blood products Antibiotics Antimicrobials Analgesics
How to follow up for lymphomas
3-4 times weekly when on chemotherapy till 6-8 cycles of chemotherapy is done ( remission)
After this 3 times monthly
LDH useful
How to follow up for leukemias
Weekly till patient is on remission
afterwards monthly for maintenance therapy
How to follow up a plasma cell myeloma
3-4 times weekly when on iv chemotherapy till remission
Monthly for maintenance after that till plateau phase
This is done for all haem malignancies
FBC