Haematology and Oncology Flashcards
What are the history and examination findings in DIC? How do you investigate it?
Signs of circulatory collapse - oliguria, hypotension, or tachycardia
microvascular/ macrovascular thrombosis - purpura fulminans,
gangrene, acral cyanosis, delrium, coma
Petechiae, ecchymosis, hematuria, epistaxis
Diagnosis = at least 1 underlying condition causing DIC + abnormal global coagulation tests (decreased PC, increased PT and PTT, elevated FDPS, decreased fibrinogen
What are the findings in essential thrombocytosis?
Investigation?
Management?
Erythromelalgia - burning pain and redness in different parts of body
Splenomegaly
Bleeding, arterial and venous thrombosis
Livedo reticularis
FBC & Blood smear -> thrombocytosis
Acute = plateletpheresis
Cytoreductive therapy if high risk and non-pregnant
Antiplatelet therapy if high risk and pregnat
what are the findings, investigation and management for myelofibrosis?
leukoerythroblastosis(nucleated Rbcs and immature wbcs) + splenomegaly = hallmarks
History and Examination
Constitutional symptoms - weight loss, night sweats, low-grade fever, pruritus, etc
Splenomegaly
Extramedullary hematopoeisis - Hematuria, spinal cord compression, focal siezures
Investigation FBC - anemia Peripheral blood smear - teardrop RBCs Bone marrow aspiration - dry tap Bone marrow biopsy - fibrosis
Management
Asymptomatic = folic acid and pyridoxine
Symptomatic = stem cell transplant
What is pancytopenia?
combination of anaemia, leukopenia, and thrombocytopenia.
What are the findings, investigation and management for polycythemia vera?
Features of thrombosis - stroke, MI, PE, DVT etc
pruritus - often aquagenic
Erythromelalgia - tender or painful burning and/or redness of fingers, palms, heels or toes
Headache, facial redness
Investigation
Hb and hematocrit - elevated
Wbc and platelets - may be elevated
JAK2 mutation screen - usually present
Management
Acute = PHLEBOTOMY + low dose aspirin
Acute High-risk thrombosis patients = cytoreductive therapy(hydroxycarbamide) + aspirin
Ongoing = busulfan
What are the key findings in TRALI?
Dyspnea, hypoxemia, pulmonary oedema
- CXR - bilateral lung infiltrates
Key findings in GvHD?
maculopapular rash is common
- diarrhoea, abdominal pain, nausea
- LFTs may be elevated
Key findings in allergic/anaphylactic reactions?
respiratory distress, angioedema, hives and itching, hypotension
Key findings in febrile non-hemolytic reactions?
negative DAT test.
- 1 degree rise in temp may have chills/malaise
Key findings in acute hemolytic reactions?
fever, abdominal pain, hypotension, tachycardia
Haemoglobinaemia, hemoglobinuria, positive DAT
Key findings in TACO?
pulmonary oedema and hypotension
Hemophilia findings and investigations?
Heamarthrosis, bleeding into muscles, mucocutaneous bleeding
pT time - normal
ApTT time - usually prolonged
Plasma factor 8 and 9 assays - decreased or absent
Findings, investigation and management for sickle cell disease?
- persistent pain in skeleton, chest, and/or abdomen
- dactylitis - swollen dorsa of hands and feet
- Others: pneumonia like syndrome, visual floaters, jaundice(indicates hemolysis of rbcs), protuberant abdomen often with umbilical hernia(enlarged spleen), pallor
Hb electrophoresis
Peripheral blood smear- nucleated red blood cells, sickle-shaped cells, and Howell-Jolly bodies(seen in hyposplenia)
Fbc
Crisis = analgesia + o2 + treatment of cause
hydroxycarbamide, L-glutamine, crizanlizumab, voxeltor, blood transfusions may sometimes be used. Bone marrow transplantation in severe cases
What are anticoagulants used for?
Give name of a drug from each group
Treat arterial and venous thrombosis
- Direct thrombin inhibitors - Dabigatran, lepirudin
- Indirect thrombin inhibitors - heparin
- Vitamin K epoxide reductase inhibitor - warfarin
- Direct Xa inhibitor - apixaban
What are antiplatelet drugs used for? Give an example from each class
Arterial disease
- Cox inhibitor - aspirin
- Glycoprotein inhibitors - eptifibatide, tirofiban
- ADP inhibitors - clopidogrel
What are thrombolytics used for?
Give examples
Arterial and venous thrombosis
Plasminogen activators - streptokinase, tenecteplase
How does alpha thalassemi present? What are the different types?
Treatment?
Microcytic anemia
- Alpha thalessemia minima - silent carrier
- Minor - mild anaemia
- Hemoglobin h disease - moderate to severe anaemia
- Hemoglobin barts disease - incompatible with life
Acute hemolytic episodes- identification of cause + folic acid
Aplastic crisis - red blood cell transfusion
Findings, investigation and management for beta thalessemia?
Microcytic anemia
Chipmunk facies
Hepatospleenomegaly and jaundice may occur
Diagnosis = increase in HbA2 on electrophoresis
Management - transfusions at times of symptomatic anaemia
What are the findings in hodgkins lymphoma?
Investigations?
painless lymphadenopathy - cervical, supraclavicular, axillary, inguinofemoral
B SYMPTOMS! (fevers, night sweats, weight loss)
Dyspnea, cough, chest pain - mediastinal involvement
uncommonly but CHARACTERISTIC: pruritus, alcohol-induced pain at involved sites
PET-CT
Biopsy - Reed-Sternberg cells
Non hodgkins lymphoma?
- non-hodgkins = NO reed-sternberg cells
- multiplel lymph nodes often involved
- extranodal involvement unlike HL
painless lymphadenopathy
b symptoms - fevers, Night sweats, weight loss)
extranodal GI involvement - Bowel obstruction
extranodal bone marrow - fatigue, easy bruising, infections
spinal cord - weakness and loss of sensation
- subdivided into b cell lymphomas (large b-cell lymphoma being the most common) and t cell lymphomas
Acute lymphocytic leukemia/ALL findings?
> 20% blasts
Tdt +ve with immunophenotyping. T(ALL)
common in children
Peripheral blood smear = leukaemic lymphoblasts
FBC: aneamia, thrombocytopenia, ELEVATED WCC but neutropenia(=infection risk)
Definitive diagnosis = biopsy which shows >20% blasts
Chronic lymphocytic leukemia/ CLL findings?
- WBC count elevated with absolute lymphocytosis
- Blood film shows SMUDGE/SMEAR cells. CL(L) -> clean -> smudge
Acute myelogenous leukemia
Investigations?
Management?
blast cells ≥20%
A in acronym -> Aeur Rods (cytoplasmic inclusions)
M in acronym -> MPO+ve
PML subtype associated with DIC and responds to treatment with all trans retinoic acid
common in older adults
FBC: may show pancytopenia
Peripheral blood smear: Auer rods
Definitive diagnosis = bone marrow biopsy and shows >20% blasts
What is Chronic myelogenous leukemia?
Investigations?
Management?
Complications?
Myeloid stem cell proliferation - productions of mature and maturing granulocytes = neutrophils, metamyelocytes, myelocytes, basophils
FBC: may show aneamia and thrombocytopenia. ELEVATED WBC!!!!
Cytogenetic testing confirms diagnosis = Philadelphia chromosome t(9,22)
Tyrosine kinase inhibitor to treat
blast crisis: blast rises to >20%
low lap score differentiates from leukomoid reaction (benign neutrophilia)
Multiple myeloma findings investigation and managment
A haematological cancer characterised by clonal proliferation of plasma cells in the bone marrow, typically associated with a monoclonal immunoglobulin in the serum and/or urine.
Bone pain(typically localised to the back) and anemia - most common
CRAB = Hypercalcemia, renal involvement, anemia, back pain
serum and urine protein electrophoresis = 1st line - paraprotein spike (IgG >35 g/L [>3.5 g/dL] or IgA >20 g/L [>2.0 g/dL] and light chain urinary excretion >1 g/day); hypogammaglobulinaemia
bone marrow aspirate and biopsy - plasma cell infiltration
whole-body low-dose CT - osteolytic lesions, pathological fractures
Under 65: Induction therapy (thalidomide + dexamethasone) + DVT prophylaxis(aspirin) + stem cell transplant. + bisphosphonates/denosumab for bone disease
Non transplant: Melphalan, prednisolon
Classic hemolysis findings?
Normocytic anemia (normal MCV) Increase in LDH Increase in indirect bilirubin Increase in reticulocyte count Urine hgb and hemosiderin (intravascular) decrease in haptoglobin
Findings and treatment for herdeitary spherocytosis?
Labs consistent with hemolytic anemia
Spherocytes on blood film
Splenomegaly
Treatment = red blood cell transfusion for symptomatic aneamia. folic acid, splenectomy if severe
What are the findings, investigations and treatment for paroxysmal nocturnal hemoglobinuria?
Hemoglobinuria(dark brown urine, typically occurring at night), budd-chiari syndrome, thrombosis
Flow cytometry for GPI anchor proteins - >1% of anchor deficient granulocytes
Urine dipstick - hemoglobinuria
Treatment = eculizumab
Findings investigation and management for G6PD deficiency?
Labs consistent with hemolytic anemia
Blood film shows heinz bodies (precipitated oxidized hemoglobin)
Ghost cells and hemighost cells from intravascular hemolysis, bite cells
avoid fava beans, oxidant drugs, and naphthalene. + give folic acid
How does pyruvate kinase deficiency present?
A cause of hemolytic anemia in a newborn. Echinocytes on blood film
Differentiate between iron deficiency anemia and aneamia of chronic disease
In iron deficiency - low iron and low ferritin(store of iron). Transferrin/TIBC is HIGH (transports iron around the body). Transferrin saturation (iron/TIBC) is low
In anemia of chronic disease - low iron and HIGH ferritin. Transferrin and transferrin saturation is low.
Associated with Chronic infections (TB, HIV) autoimmune disorders like RA, malignancy.
How does aplastic aneamia present?
May present with neutropenia, anemia or thrombocytopenia
Bone marrow biopsy - hypocellular without abnormal cells
RFs - radiation and drugs, PNH
Immunosuppressants + stem cell transplantation
Differentiate between the different causes of megaloblastic anemia and how they present
Vitamin B12 - neurological symptoms, increase in methylmalonic acid and homocysteine
Folate deficiency - no neurological symptoms, normal methylmalonic acid. Raised homocysteine. If both B12 and folate deficiency, treat B12 first to prevent SACD
Agents or mutations that impair DNA synthesis:
azathioprine
cytotoxic chemotherapy
Folate antagonists - methotrexate
Bone marrow cancers - myelodysplastic syndrome
Treatment for b12 and folate deficiency?
B12 = IM hydroxycobalamin Folate = oral supplements
key leukemia symptoms?
fatigue - anemia
easy bleeding -thrombocytopenia
infections - neutropenia
pain and tenderness in bones due to blast production in acute leukemias
hepatomegaly, spleenomegaly, lymphadenopathy variable
What is tumour lysis syndrome?
What are the features?
Complication of cancer treatment/chemo
- Kidney Failure and Hypocalcemia - due to released phosphate that forms crystals
- Released Uric acid -> Gout
- Released Potassium -> Hyperkalemia and arrhythmias
What are the findings in TTP?
HUS + fever and neurological manifestations
Hemolytic ureamic syndrome = triad of Hemolytic anemia(low Hb, high bilirubin), uremia(AKI- may show rise in creatinine, proteinuria, hematuria) and thrombocytopenia.
Usually following Diarrhea episode!! which is usually bloody!!! and due to shiga-toxin producing e.coli.
