Hematology

Question 1

1. Introduction: What are hematological Malignancies
2. Epidemiology
3. Aetiology
4. Genetics
5. Diagnostic investigations
6. Management

Answer 1

1. Introduction: hematological Malignancies are clonal disease that derive from hemopoietic tissues such as blood, lymph nodes, bone marrow. They are usually derived from a single cell. They include leukemia, lymphoma, myelodysplastic syndrome(MDS), myeloproliferative neoplasm(MPN).
   Most times the tissue of origin has undergone genetic alteration.
2. Epidemiology: they account for approx 7% of all cancer. In males-9% while females-7%.
3. Aetiology: genetic and environmental factors influences the risk of developing Malignancy.
   Genetics: aka chromosomal breakage syndrome. They include: down syndrome, bloom syndrome, fanconi’s anemia, ataxia telangiectasia, neurofibromatosis, klinefelter syndrome, wiskott-abrinch syndrome.
   Environmental factors: exposure to chemicals and radiation, drugs(alkylating drugs which are used for treatment of cancer such as procarbazine, chrolambucil, mephalan)
   Infective agents: viruses such as human T cell lymphotropic virus type 1(HTLV-1) which causes adult T cell leukemia /lymphoma
   Epstein Barr virus causes hodgkin and non-hodgkin lymphoma (endemic burkitt lymphoma)
   Human herpes virus 8(HHV-8)which causes kaposi sarcoma and primary effusion lymphoma
   HIV-unknown site of lymphoma
   Bacteria: H. Pylori - mucosa associated lymphoid tissue lymphoma
   Protozoa: Malaria alters host immunity predisposes to endemic burkitt lymphoma
4. Genetics: oncogenes and tumor suppressor genes are involved in the development of cancer
   Oncogens: proto oncogenes become oncogenes by gain of function mutation. T processes involved are translocation(seen in CML(9;22), AML(15;17), ALL(12;21) follicular lymphoma(14;18) and duplication(chromosome 8,12,19,21 Y e.g trisomy 21 seen in CLL.
   Tumor suppressor genes: they become cancer causing genes by loss of function mutation. Involves two processes: point mutation(FLT-3 in AML) and deletion(involves chromosome 5, 6,7,11,20 and Y) hypoploidy is seen in ALL
5. Diagnostic investigations:
   +karyotyping
   +immunohistochemistry
   +PCR
   +flow cytometry
   +DNA micro-array
   +fluorescent in situ hybridization(Fish)
   + gene sequencing
6. Management: definitive investigations include( FBC, blood film, bone marrow biopsy, cytochemistry, cytogenetics, immunophenotyping, molecular genetics) and supportive
   Definitive treatment: combination cytotoxic chemotherapy, surgery, radiation

Question 2

CHRONIC MYELOID LEUKEMIA
+definition/introduction /epidemiology
+pathogenesis/risk factors
+clinical features/physical examination
+phases
+investigations
+treatment
+differentials

Answer 2

+CML can be defined as a clonal disorder of hemopoietic Stem cell characterised by granulocytosis, thrombocytosis, basophilia and splenomegaly. It is a myeloproliferative disorder that affects the granulocytic cell line. More common in females and affects adults more
+ translocation between chromosome 9 and 22 forming tyrosine kinase. Exposure to ionising radiation is a risk factor
+ fatigue, weight loss, night sweat, anorexia, splenomegaly, pallor, dyspnoea, tachycardia, Abdominal pain and swelling, bleeding, epistaxis and menorrhagia. On physical examination; pallor, splenomegaly and sternal tenderness
+chronic phase, accelerated phase and blast transformation phase
+definitive and supportive treatment
+ PILE

Question 3

APLASTIC ANEMIA
+definition
+how can aplastic anemia be diagnosed
+epidemiology
+aetiological classification
+drugs and virus associated with idiosyncratic acquired AA
+pathogenesis
+clinical features
+investigations
+differentials
+laboratory diagnosis and bone marrow findings
+severity
+treatment

Question 4

Non-hodkins lymphoma
+introduction
+pathogenesis
+aetiology
+WHO classification
+clinical presentation
+laboratory diagnosis
+Staging
+prognostic factors
+types of NHL
+treatment

Question 5

ACUTE MYELOID LEUKEMIA
+what is leukemia
+what is acute myeloid leukemia
+it is also known as?
+what does acute mean in leukemia
+epidemiology
+pathogenesis
+Causes of AML
+classification
+differences between myeloblast and lymphoblast
+clinical features
+diagnostic investigations
+reason for doing bone marrow aspiration
+special stains used for AML

Answer 5

+leukemia is a clonal disorder characterised by malignant white cells
+ proliferation