Hematology System Flashcards
(14 cards)
What is anaemia?
What are the causes of anaemia?
What are the names of the different forms of anemia?
Reduced absolute no. of circulating Blood cells. Ie its a reduction in one or more of the major RBC measurments of a blood count: haemoglobin, hematocrit, or RBC count.
Hemoglobin concentration: The major o2 carrying pigment in the blood.
Hematocrit: The 5 of a sample of blood occupied by intact RBC
RBC count: no. of RBC contained in a specified colume of whole blood
Causes of anemia?
Causes of anemia can be classified according to measurments of RBC size. Normal RBC volume =80-100 femtolitres.
-The RBC larger than the nucleus of a small lymphocyte are considered MACROLYITIC
while those that are smaller are MICROLYTIC
Forms of anemia:
- Microlytic- iorn deficiency anemia
- microlytic- thalassemia
- thalassemia- Beta
- thalassemia- Alpha
- macrolytic- B12 deficiency anaemia
- macrolytics- pernicious anaemia
- macrolytic (megablastic)- folate anaemia
- normacytic
Microlytic- iron deficiency anaemia What is it causess? Risk factors? Histroy? test/investigations Management? complications? prognosis?
-iron is critical in some enzymes eg cytochrome system
-without iron cells lose capacity of energy metabolism
can result from?
-dietry lack
-impaired absorption
-increased requirements
-chronic blood loss
This anemia only appeaers when iron stores are depleted completely & accompanied by low serum iron, ferretin and transferrin.
Poor iron stores result in impaired Hb synthesis and microlytic anaemia.
Risk factors:
- pregnancy
- vegan diet
- celiac disease
- NSAIDS use
History:
-fatigue, plica( want to eat random shit), hair loss, dysphagia, restless leg syndrome, gastric difficulties.
tests:
- Hb low
- platelet count elevated
- MCV <80 (mean corpuscular volume- average size of RBC)
- serum iron low
- ferretin low
- transferrin saturation low (their the guys that transport iron in blood)
Management:
- Iron supplementation
- Vit C (increases absorption as it reduces Fe3= to Fe2+)
- blood transfusion
Complications (rare):
- cognitive impairment
- heart failure
- infection from transfusion.
Prognosis:
Depends on underlying cause- pregancy good, cancer bad.
Microlytic- Thalassemia
pathophysuiology
Thalassemia is inherited disorder:
we have:
-a pair of genes on chromosome 16 –> alpha subunit
-1 gene on chromosome 11–> B subunit
-a complete Hb molecule has 4 subunits
-Thalassemia occurs when genes fail to produce a protein (ie shortage of one subunit)
-If B globin genes fail- beta thalassemia
If- A globin genes fail- A thalassemia.
Thalassemia- Beta Risk factors History/ exam test/ investigations Managment Complications prognosis
Risk factors:
- family history
- mediterranean, india, southeast asia etc
History:
- Lethargy
- abdominal distention
- failure to gain weight
- low height and weight
- large head
- chipmunk face
- misaligned teeth
- jaundice
Tests/ Investigations: -Hb A decrease Hb- F elevated -x-ray of skull -FBC- (full blood count)- microlytic anaemia
Management:
- Transfusion
- possible spenectomy (spleen removal)
Complications: iron overload Prognosis: Trait- normal Intermediate- changes that can be managed Major- fatal in first few yrs of life.
Thalassemia- Alpha Risk factors History/ exam test/ investigations Managment Complications prognosis
-same risk factors History is different in that: -fatigue -dizziness -shortness of breath -splenomegaly -gallstones -growth retardation -childhood or young adult
tets/Investigations:
- FBC-microlytic anaemia
- Hb low
- RBC increased
- serum iron and ferretin normal or elevated
Management:
- red blood cell transfusion
- splenectomy
- decrease iron in diet
Complications: iron overload
prognosis:
silent- asymptomatic
Hb H- variable
Macrolytic (magsloblastic) -B12 Deficiency anaemia
B12 deficiency causes impaired DNA synthesis due to the reduced availablity of FH4.
- Vit B12 is eessential for DNA synthesis + related to folate metabolism
- Imairs conversion of something into methionine which is important to neural function.
- Vit B12 ans folate integral to normal haematopoises and bone marrow function.
Risk factors:
- > 65
- gastric surgery
- terminal ilieum disease
- vegan diet
History:
- Parasthesia (tingling sensation)
- cognition impairment or dementia
- impaired neurological sensors (numbness, poor balance, co-ordination, tachycardia, pallor)
Test/ investigations:
- FBC- MACROlytic anaemia
- B12 levels low
Management:
- Blood transfusion
- diuretic
- oral folic acid
- multivitamins
- B12
Complications:
- neurological defecits
- haematological defecits
Macrocytic- Pernicious anaemia
Resulting from an auto-immune attack on the gastric mucosa.
Caused by an autoimmune- gastritis and failure of intrinisx factor production, leading to vit 12 deficiency.
Macrolytic Foliate Anemia Aetilogy pathophys risk factors history/ examination test/ investigations
megaloblastic anaemia without neuropathy is the classic manifestation of folate deficiency
Aetiology:
-consumption of un-fortified cereals, poor diets, goats milk.
Patho: Deficiency affects DNA synthesis. This causes reduced cell dividions, so the rapidly dividing cells of bone marrow (resulting in anaemia), neutropenia, and thrombocytopenia. leading to inflammatory changes.
Risk factors:
- low dietary folate
- > 65
- Alcoholism
- pregnant or lactating
History:
- prolonged diarrhoea
- headache
- loss of apetite
- fatigue
- SOB
- dizziness
- Pallor
- tachycardia
- signs of heart failure
Management:
- Folic acid supplementation
- tx underlying condition
- packed RBC transfusion
Complications:
- neural tube defects in fetus
- progression of neuropathy
prognosis:
- normalise after 8 weeks of therapy
- avoid alcohol or other drugs
Normacytic- Anaemia of chronic disease/ chronic inflammation
- anaemia of the inflammatory response from chornic infection or chronic immune activation.
- when heaps of IL’s are being released it makes EPO less effective and effects its secretions.
- Also prevents release of iron stores
- Inflammatory cytokines also promotes production of WBC, thus there are fewer stem cells to differentiate into RBC
-ferretin levels low
Hodgkins/ non-Hodgkins lymphomas
Whats the diff?
Hodgkins- malignancy arising frin mature B cells
Non-Hodgkins lymphomas- malignancies of the lymphoid system
History:
- Lymphadenopathy
- splenomegaly
- normal symptoms of cancer (night sweating, weight loss etc)
Tests/ invetigations:
- FBC- thrombocytopenia
- lymph, skin, bone marrow biopsy
- ESR elevated
management:
- radiotherapy
- chemotherapy
Complications:
-radio and chemo side effects
Prognosis:
HL- 85-95% cure rate
NHL- variable, depending (20-50%)
Leukaemia
What is it?
What are the different types?
Leukemia is the unregulated growth of leukocytes in bone. Ie increasing or decreasing no. of circulating leukocytes in blood and marrow failure –> therefore decrease RBC, infections (dec. mature WBC, and hemorrhage (dec. platelets); leukemic cell infiltrates in liver, spleen and lymph nodes.
Different types:
Acute leukemia
-acute myelogenous leukemia
-acute lymphoblastic leukemia
Chronic Leukemia
- chronic myelogenous Leukemia
- chronic lymphocytic leukemia
Acute Leukemia
describe in general what it is, then go into the 2 types.
-rapid onset
-short survival time (with no treatment)
ALL is characterised by an overproduction of immature white blood cells, called lymphoblasts or leukaemic blasts. These cells crowd the bone marrow, preventing it from making normal blood cells. They can also spill out into the blood stream and circulate around the body. Due to their immaturity, these cells are unable to function properly to prevent or fight infection. Inadequate numbers of red cells and platelets being made by the marrow cause anaemia, and easy bleeding and bruising.
Symptoms:
-Anemia–> fatigue
-absence of mature WBC–> fever
-low platelets–> bleeding
-bone pain- tenderness
-CNS effects- headache, vomiting, nerve palsies.
- Acute Myelogenous Leukemia
ALL is characterised by an overproduction of immature white blood cells, called lymphoblasts or leukaemic blasts. These cells crowd the bone marrow, preventing it from making normal blood cells. They can also spill out into the blood stream and circulate around the body. Due to their immaturity, these cells are unable to function properly to prevent or fight infection. Inadequate numbers of red cells and platelets being made by the marrow cause anaemia, and easy bleeding and bruising.
Clinical features:
- Accumulation of leukemic cells –> decrease normal hematopoetic cells–> anemia, thrombocytopenia, leukopenia (fatigue, bruising, internal hemorrages/ bleeding, infections fever)
- soft tissue infiltration causes hepatosplenomegaly and enlarge lymoh ndes
- CNS manifestation (meningeal spread): headache, vomiting and nerve palsies
-85% in adults, 20% in children
prognosis:
Untreated: <2 months
-60%= complete remission with chemotherapy, 30% remain free from disease for 5 yrs
-50-60% of patients that undergo marrow transplant appear to be cured.
Acute Lymphoblastic Leukemia
Acute lymphocytic leukemia (ALL), also called acute lymphoblastic leukemia, is a cancer that starts from the early version of white blood cells called lymphocytes in the bone marrow (the soft inner part of the bones, where new blood cells are made).
-a progressive medullary and extramedullary accumulation of lymphoblasts that lack potential for differentiation and maturation.
Clinical features:
-similar to those in AML, but accumulated leukemic cells are immature lymphoblasts.
Prognosis:
-Untreated = 90% of children with favourable prognosis, majority –> complete remission
