Oncology & Hemat Flashcards
1
Q
G6PD Def
A
(X-linked recessive)
Bite & blister cells
Heinz bodies
reduced NADPH levels
leads to a lack of the enzyme glucose-6-phosphate dehydrogenase, making red blood cells vulnerable to oxidative stress, which can cause premature breakdown (hemolysis) and hemolytic anemia.
common in males, present in newborns, childrens, and adults
Triggers: Hemolysis occurs when exposed to:
Foods: Fava beans (favism), tonic water, blueberries
Medications: Antimalarials (e.g., primaquine), sulfa drugs, aspirin (high doses), nitrofurantoin, methylene blue
Infections or stress (e.g., severe illness)
2
Q
G6PD Def causes
A
Drugs- cipro, sulphur, Premaquine
can manifest as hemolytic anemia, characterized by red blood cell breakdown, causing symptoms like pallor, jaundice, dark urine, fatigue, and potentially an enlarged spleen, triggered by factors like infections, certain medications, or fava bean consumption
3
Q
APML
A
DIC
T-15 17
Treatment - ATRA
4
Q
Acute leads poisoning features
A
abdominal pain, Neurological, blue lines on gum margines,
baso
5
Q
Basophilic stippling
A
small, dark-blue/purple granules -ribosomal RNA aggregates
Lead Poisoning, Thalassemia, megaloblastic anemia , sideroblastic anemia, Myelodysplastic Syndromes
6
Q
cold autoimmune haemolytic anaemia causes
A
Action - IgM autoantibodies cause RBC agglutination
causes - Mycoplasma pneumoniae, Epstein-Barr virus, lymphoma,
7
Q
warm autoimmune haemolytic anaemia causes
A
IgG
SLE, rheumatoid arthritis, CLL, lymphoma, Epstein-Barr, solid tumors, alpha-methyldopa, transplants
8
Q
CLL worse prognostic factors
A
TP53 (tumor suppressor gene) - del 17p,
9
Q
Bone Mets in descending order
A
cancers
1. prostate
2. breast
3. lung
common sites of deposition
spine
pelvis
ribs
skull
long bones
10
Q
methemoglobinemia causing drugs
A
dapsone, local anesthetics like benzocaine and lidocaine, poppers
congenital causes - enzyme cytochrome b5 reductase, NADH MHB reductase ,
Normal hemoglobin has iron in a reduced state (ferrous, Fe2+), while methemoglobin has iron in an oxidized state (ferric, Fe3+).
Symptoms of Methemoglobinemia:
Cyanosis
Fatigue and Weakness: A general feeling of tiredness and lack of energy.
Headache: Pain in the head.
Shortness of Breath: Difficulty breathing.
Nausea and Vomiting: Feeling sick to the stomach and throwing up.
Dizziness and Lightheadedness: Feeling unsteady or faint.
Rapid Heart Rate: An increased heart rate.
Confusion and Lethargy: Disorientation and drowsiness.
Seizures: Uncontrolled muscle spasms.
Loss of Consciousness: Fainting or becoming unconscious.
Chocolate-Brown Blood: A telltale sign of methemoglobinemia is the presence of dark, brown-colored blood
11
Q
CML mech
A
chromosomal translocation (t(9;22)) that creates the BCR-ABL1 fusion gene
High WBC, Basophelia, splenomigaly
asymptomatic at presentation in approximately 50% of patients. Symptoms, if present, typically include malaise, fever, weight loss, abdominal discomfort, and night sweats. Splenomegaly is the most common physical finding; elevated WBC count
Progression:
Chronic phase (stable, treatable) → Accelerated phase (worsening symptoms) → Blast crisis (aggressive, like acute leukemia).
Epidemiology:
Accounts for ~15% of adult leukemias.
Typically diagnosed in middle-aged and older adults (median age ~64).
Diagnosis
Blood Tests:
High WBC count (often >100,000/µL).
Low RBCs (anemia) and abnormal platelets.
Bone Marrow Biopsy: Confirms excess immature cells (myeloid precursors).increased numbers of granulocytes, megakaryocytes, and sometimes eosinophils and basophils
Genetic Testing:
PCR or FISH detects BCR-ABL1 fusion gene.
Cytogenetics identifies the Philadelphia chromosome (t(9;22)).
Treatment
1. Tyrosine Kinase Inhibitors (TKIs) – First-Line Therapy
These drugs block BCR-ABL1 and are highly effective:
Imatinib (Gleevec) – First TKI, >80% long-term survival.
2nd-gen TKIs (Dasatinib, Nilotinib, Bosutinib) – Used if resistance/intolerance to imatinib.
3rd-gen (Ponatinib) – For T315I mutation (resistant to other TKIs).
- Other Treatments
Stem Cell Transplant – Only curative option, but high risk; reserved for TKI-resistant or blast crisis cases.
12
Q
Hereditary Angioedema identification
A
C1 - acute attack
C4 - in between attracks
13
Q
Leukemia connected witrh DIC
A
acute promyelocytic leukemia
14
Q
Li Fraumeni syndrome
A
Germenite mutation to P53
15
Q
pentade of TTP
A
Fever, Anemia (microangiopathic hemolytic anemia), Trombocytopenia, Renal dysfunction, and Neurologic abnormalities
rare and potentially life-threatening blood disorder characterized by the formation of blood clots in small blood vessels, leading to low platelet counts, red blood cell breakdown (Schistocytes - key indicator of MAHA), and potential organ dysfunction
treatment - Plasma Exchange, prednisone, help to slow or stop the body from forming antibodies against the ADAMTS13 enzyme, Rituximab - anti-CD20 monoclonal antibody that helps to reduce the number of B cells, which are responsible for producing the antibodies that cause , Caplacizumab
Congenital TTP: In cases of congenital TTP, where the ADAMTS13 enzyme is deficient, fresh frozen plasma infusions may be used to replace the missing enzyme
16
Q
TTP mechanism of action
A
congenital or acquired absence/decrease of the von Willebrand factor-cleaving protease ADAMTS13
17
Q
Wiskott-Aldrich syndrome
A
primary immune deficiency (combined B & T), eczema, irritated skin, thrombocytophenia
X linked ressesive
18
Q
Essential thrombocytosis
A
JAK2, CALR, MPL
myeloproliferative neoplasms
overlap with CML, PRV, and myolofibrosis
19
Q
Tthrombocytois causes
A
stress
20
Q
- oestrogen in post menapause
- aromataze inhibitors
- Serm
A
- andregen
- anastrezole and lestrazole
- act as a oestragen receptor antagonist
21
Q
tumor lysis syndrome treatment
A
Rasburicase should not be given with Allopur
22
Q
tumor lysis syndrome features
A
high K+
High Ph+
low Ca+
23
Q
cisplatin mechamism
A
cross linking DNA hypomagnisemia
24
Q
Acute intermittent porphyria
A
inherited metabolic disorder caused by a deficiency of the enzyme porphobilinogen deaminase
25
Waldenström macroglobulinemia
abnormal protein (IgM) > 50
affects B-cells
non-Hodgkin lymphoma
generalized muscle weakness, fatigue
Hyperviscosity - retinopathy, headache, vertigo, mucosal bleeding, seizures and coma
Bence Jones proteinuria in 10% of cases
Constitutional symptoms, splenomegaly, anemia and lymphadenopathy
26
Aplastic anemia treatment
1. Blood Transfusions
2. Antibiotics
3. Immunosuppressive Therapy ATG. ALG, Cyclosporine
4. BMT
5. Medications - Growth factors, Eltrombopag, Hypomethylating agents
27
polysythaemia Rubra Vera causing cancers
Key Features:
Hyperviscosity → Increased risk of thrombosis (stroke, DVT, MI).
Secondary complications: Pruritus (after warm showers), erythromelalgia (burning pain in hands/feet), splenomegaly.
Risk of progression: To myelofibrosis (10–20%) or AML (1–5%) over time.
a is a chronic myeloproliferative neoplasm (MPN), disorder where too many red cells are produced in the bone marrow, without any identifiable cause.
common in males over 40
blood test - erythrocytosis, leukocytosis, thrombocytosis, Increased Hematocrit & Hemoglobin, Decreased Erythropoietin
Major Criteria:
Hemoglobin >16.5 g/dL (men) or >16.0 g/dL (women) OR
Hematocrit >49% (men) or >48% (women).
Bone marrow biopsy showing hypercellularity with trilineage growth.
JAK2 V617F or exon 12 mutation.
Minor Criterion:
Low serum erythropoietin (EPO) level.
fatigue, headaches, dizziness, and itching, especially after a warm bath, as well as an increased risk of blood clots and bleeding. risk factor for Budd-Chiari syndrome (BCS)
✅ PV = JAK2 mutation + high Hct + low EPO.
✅ Phlebotomy + aspirin for low-risk; hydroxyurea for high-risk.
✅ Watch for pruritus, erythromelalgia, and thrombosis.
✅ Ruxolitinib if refractory to hydroxyurea.
28
ITP platelet receptor
gp -iib/iiia , ib -v9
29
Treatment for sideroblastic anemia
pyridoxine (vitamin B6), blood transfusions, iron chelation
30
ring sideroblasts anaemia causes
gentical, medication, alcohol
31
post splenectomy blood film
Howell-Jolly bodies, Pappenheimer bodies, target cells, and acanthocytes
potential leukocytosis and thrombocytosis
After a splenectomy (spleen removal), individuals are at increased risk of infections, overwhelming post-splenectomy infection (OPSI), bleeding, blood clots, or pneumonia.
32
CML translocation gene
Philadelphia chromosome, 9-22
33
Von Willebrand disease
1. impact
2. treatment
3. types
1. Mucocutaneous Bleeding, bleeding from gums, menohrragea , Easy bruising:, Frequent nosebleeds, GI/ Urinary bleeding
autosomal dominant, caused by a deficiency or dysfunction of von Willebrand factor (VWF), a protein crucial for blood clotting, either quantitatively or qualitatively
2. Desmopressin (DDAVP), tranexamic acid, Factor Replacement Therapy,
3. Type 1: Partial deficiency of VWF - mild or no symptoms
Type 2: Qualitative defects in VWF
Type 3: Complete deficiency of VWF - near-total absence of von Willebrand factor (VWF) and a significant deficiency in factor VIII (FVIII), leading to a severe bleeding disorder with symptoms like easy bruising, prolonged bleeding, and potential for hemarthrosis and muscle hematomas - prolonged bleeding time, prolonged APTT
should be informed to haematologist before surgery, dental extraction, new medication - NSAIDS, aspirin
34
hereditary spherocytosis diagnosis test
EMA binding
MCHC high
35
Beta thalassemia major (Cooley's anemia) patho
two mutated beta-globin genes
36
Beta Thalassemia Trait (Minor) signs
3.5< HbA2, minor anaemia
microcytosis is dispropotionately low compared with anemia , bull's-eye" or "target cells
asymptomatic
37
Alpha Thalassemia Major (Hemoglobin Bart's Syndrome) patho
2. present as
absence of all four alpha-globin genes
2. fetal hydrops, stillbirth
3. chromsome 16
38
1. Acute intermittent porphyria (AIP) signs
2. diagnosis
1. abdo, neuro, psych, inherited metabolic disorder
2. porphyrin precursors in the urine
39
Burkitt lymphoma gene mutation
translocation of the MYC - proto-oncogene- 8, 14 chromozome
- stary sky appearance,
- E B V associated
- three clinical forms: endemic, sporadic, and immunodeficiency-associated.
40
Burkitt lymphoma treatment
2. treatment for tumor lysis
Chemotherapy,
2. rasburicase
3.
41
general features of hemolytic anemia
anaemia, reticulocytosis, low haptoglobin, raised LDH, blood film - spherocytosis, reiculocytosis, positive coombs test,
42
criteria for multiple myeloma,
C: alcium: Elevated calcium levels in the blood.
R: enal insufficiency
A: nemia: Low red blood cell count.
B: one disease: Bone damage or fractures.
43
MGUS (Monoclonal Gammopathy of Undetermined Significance
M-protein or paraprotein
monoclonal paraprotien band <30
Plasma cells <10% on bone marrow
No CRAB syndromes
Asymptomatic premalignant state
44
Follicular lymphoma (FL) gene
14-18
Slow-growing, considered an indolent lymphoma, Arises from B-lymphocytes
Painless swelling in lymph nodes, often in the neck, armpit, or groin. Other symptoms can include fatigue, loss of appetite, and unexplained aches and pains.
Can sometimes transform into a faster-growing lymphoma.
45
Cisplatin mechan
cause low magnesium,
Crosslinking DNA
Cause acute tubular nectrosis -
46
Sickle cell long term treatment
hydroxicarbomide
47
CLL blood picture
Smudge cells
dentification of small, mature B lymphocytes with a characteristic morphology and immunophenotype in peripheral blood, bone marrow, and lymph nodes.
Early Stage (Often Asymptomatic):
Detected incidentally on routine blood tests (↑ lymphocytes).
Mild fatigue, enlarged lymph nodes ("rubbery" nodes in neck/armpits/groin).
Advanced Stage:
B symptoms: Fever, night sweats, weight loss.
Anemia (fatigue, pallor) or thrombocytopenia (bleeding/bruising).
Recurrent infections (pneumonia, herpes zoster).
Hepatosplenomegaly (enlarged liver/spleen → abdominal discomfort).
Diagnosis
Blood Tests:
Absolute lymphocytosis (>5,000 B-cells/µL for ≥3 months).
Flow cytometry: Confirms CD5+, CD19+, CD23+ B-cells (CLL immunophenotype).
Bone Marrow Biopsy (not always needed).
FISH/Cytogenetics:
Del(13q) (good prognosis), Trisomy 12 (intermediate), Del(17p)/TP53 mutation (high-risk) - Avoid chemotherapy (resistance).
Use BTK inhibitors (Ibrutinib) or Venetoclax-based regimens.
IgHV Mutation Status:
Mutated IgHV → better prognosis.
Unmutated IgHV → more aggressive.
Staging (Rai or Binet Systems)
Rai Stage Features Risk Group
0 Lymphocytosis only Low
I Lymphocytosis + lymphadenopathy Intermediate
II Lymphocytosis + hepatosplenomegaly Intermediate
III Lymphocytosis + anemia (Hb <11 g/dL) High
IV Lymphocytosis + thrombocytopenia (Plt <100K) High
Binet System (based on lymphoid areas involved + cytopenias).
Treatment
1. Watch & Wait (for Asymptomatic Early Stage)
No survival benefit from early treatment.
Regular monitoring (CBC, clinical exams).
2. When to Treat?
B symptoms, progressive lymphadenopathy, cytopenias (Hb <10, Plt <100K), or rapid lymphocyte doubling time (<6 months).
3. First-Line Therapies
Targeted Agents (Preferred for Most Patients):
BTK Inhibitors (Ibrutinib, Acalabrutinib) – Blocks B-cell signaling.
BCL-2 Inhibitor (Venetoclax) + Anti-CD20 (Obinutuzumab/Rituximab).
Chemoimmunotherapy (for Fit, Non-High-Risk Patients):
FCR (Fludarabine + Cyclophosphamide + Rituximab) – If IgHV-mutated.
BR (Bendamustine + Rituximab) – Less aggressive option.
4. High-Risk Disease (Del17p/TP53 mutation)
48
Haptoglobin levels in hemolytic anemia
1. mechanism
low
49
IgG4 disease
Chronic, immune-mediated condition that can affect multiple organs, often presenting with tumor-like masses and fibrosis
Retroperitoneal fibrosis (RPF)
50
Trali features
2. management
low saturation, high RR, high HR, low BP, raised temp,
2. stop transfusion, IV fluids, Oxygen, supportive care
51
Teardrop poikilocytosis/ dacrocytosis along with high platelet & WBC
Myelofibrosis
52
Cyclophosphamide
Cross linking DNA
53
Methyotrexate action
inhibit DHF reductace and Thimiddilate synthesis
cause myeolo suppression, liver & lung fibrosis, mucocytosis
54
CML treatment
Imatinib - tyrosine kinase inhibitor
55
drugs can cause peripheral neuropathy
Platinum drugs, such as cisplatin, carboplatin, and oxaliplatin
Taxanes, such as docetaxel, paclitaxel, and cabazitaxel
Vinca alkaloids, such as vinblastine, vincristine, vinorelbine, and etoposide
56
Waldenström macroglobulinemia
Waldenström macroglobulinemia is a rare, indolent B-cell lymphoma characterized by:
Lymphoplasmacytic infiltration of bone marrow
Monoclonal IgM paraprotein production (≥3 g/dL)
Symptoms related to IgM effects (hyperviscosity, neuropathy, cryoglobulinemia)
Pathophysiology
MYD88 L265P mutation (90-95% of cases)
CXCR4 mutations (30-40%; associated with treatment resistance)
Bone marrow infiltration by small lymphocytes + plasmacytoid cells
IgM-Related Symptoms
Hyperviscosity syndrome (blurry vision, headache, epistaxis)
Peripheral neuropathy (IgM anti-MAG antibodies)
Cold agglutinin disease (hemolytic anemia)
Cryoglobulinemia (Raynaud's, purpura
Diagnostic Criteria (WHO/2016)
IgM monoclonal gammopathy (any level).
Bone marrow infiltration by ≥10% lymphoplasmacytic cells.
MYD88 L265P mutation (supports diagnosis but not required).
Differential Diagnosis
Multiple myeloma (IgM type) (rare; look for CRAB symptoms).
Chronic lymphocytic leukemia (CLL) (CD5+, CD23+).
Marginal zone lymphoma (MZL) (different immunophenotype).
Test Findings in WM
Serum protein electrophoresis (SPEP) IgM spike (≥3 g/dL suggests WM).
Immunofixation Monoclonal IgM (κ or λ light chain).
Bone marrow biopsy Lymphoplasmacytic infiltration (CD20+, CD5–, CD10–).
MYD88 L265P testing Positive in ~90%.
Serum viscosity Elevated if >4 cP (normal: 1.4-1.8).
Cryoglobulins May be present (Type I or II).
First-Line Therapy
Regimen Mechanism Notes
Rituximab + bendamustine Anti-CD20 + alkylator Preferred for most patients.
Ibrutinib (BTK inhibitor) Targets MYD88 pathway Best for MYD88-mutated/CXCR4 wild-type WM.
Dexamethasone + rituximab + cyclophosphamide (DRC) Chemoimmunotherapy Alternative for non-mutated cases.
C. Treatment for Hyperviscosity
Plasmapheresis (urgent if symptomatic).
Hydration + rituximab (avoids IgM flare).
57
CLL diagnosis
Immunophenotyping
other presentations - persistent lymphocytosis (at least 5 x 10^9/L B lymphocytes in the peripheral blood for at least 3 months) and confirmation of B-cell clonality using flow cytometry. Peripheral Blood Smear, Smudge Cells
58
APML
treatable, type of acute myeloid leukemia (AML) characterized by the overproduction of immature white blood cells called promyelocytes, often leading to bleeding and clotting problems 15,17
Diagnosis: APML is diagnosed through blood tests and bone marrow biopsies
pancytophenia
59
cancer connected to
1. EBV
2. HTLV 1
3. HIV
4. H Pylori
5. malaria
1. Hodgkin's, Burkitts lymphoma, nasopharangial carcinoma
2. Adult T cell leukaemia/lymphoma
3. high grade B cell lymphoma
4. MALT
5. Burkitt's lymphoma
60
leukemoid reaction
increase in presence in immaure cells, myloblasts, promyoloblasts]
infection, bonen marrow,
61
Cryoglobulinemia
1. type 1
2. treatment
1. associated with multiple myoloma,waldenstrom
2. plasmapheris
62
differentiate CML from leukemoid reaction
Leukemoid reaction - high laps score, toxic granules, dolls bodies in WBC, left shift in Neutrophils
63
Taxenes , docetaxens actiom
prevents microtubule depolymerization & disassebly decresing free tubulin
64
Multiple Myeloma
Neoplastic proliferation of a single plasma cell lineage aka M-protein (Usually of the IgG or IgA type)
Paraprotein >30g/L
Plasma cells >10% on bone marrow
CRAB SYMPTOMS
prognostic criteria -
1. Clinical Factors - stage, age, blood test results(Hemoglobin Level, Serum Calcium, Renal Function, M-Protein Levels)
2. Blood test results - beta-2-microglobulin, albumin, LDH, creatinine, CRP),
3. genetic abnormalities (chromosome 13 (del(13))), translocations t(4;14) and t(14;16) High plasma cell proliferation (PCLI)
4. response to treatment
65
Graft-versus-host disease
skin biopsy
bone marrow macrophagous
66
Cryoglobulinemia
Hepatitis C virus, recuded C3/4
67
Common variable immunodiefficiency
low antibody levels, recurrent infections,
68
APML treatment
All trans retinoid acid
69
CML Vs CLL
CML occurs in myeloid cells, while CLL occurs in lymphocytes
70
Associations of Thymoma
Pure red cell aplasia, SLE, dermatomyocitis, SIADH, MG
71
common lung cancers
1. smokers
2. non smokers
1. small cell lung cancers
2. adenocarcinoma
72
Essential thrombocytosis
Platelet >600
73
wafferin induced skin nectrosis
protien C diefficiency
doesnt inactivate factor 5a,8
74
paroxisimal nocturnal heam urea connection
AML
75
If MG suspected next step
CT to find mediastinal mass
76
drug to Decrease the incidence of infection resulting from neutropenia
filgrastim
77
Coproporphyrinogen III
common test for lead toxi
78
Hereditary spherocytosis
An inherited blood disorder causing red blood cells to become spherical and fragile, leading to their premature destruction and resulting in anemia, jaundice, and splenomegaly.
autosomal dominant
Common complications:
cholelithiasis, haemolytic episodes, and aplastic crises
Anemia: Jaundice, Splenomegaly, Gallstones,
79
Autoimmune hemolytic anemia (AIHA
is the most common autoimmune condition associated with chronic lymphocytic leukemia (CLL) and small lymphocytic lymphoma (SLL).
80
hodgkin lymphoma
often presents with painless swelling of lymph nodes, especially in the neck, armpits, or groin, along with symptoms like fever, night sweats, and unexplained weight loss., Pain with Alcohol, more common in young adults
Diagnostic Tests: Lymph Node Biopsy, Reed-Sternberg Cells,
81
Sickle cell disease
characterized by abnormally shaped, rigid red blood cells that block blood flow, leading to pain, anemia, and other complications
autosomal recessive pattern
increased risk of infections from encapsulated bacteria like Streptococcus pneumoniae, Haemophilus influenzae, and Neisseria meningitidis due to functional asplenia and immune deficiencie
mortility- sickle cell disease
valine to glutamic acid mutation
82
AML
prognostic markers - cytogenetic and molecular mutations
Mutations in genes like NPM1 and CEBPA, Cytogenetics - t(8;21) and inv(16) are associated with a better prognosis
Mutations in genes like FLT3 (especially FLT3-ITD), TP53, deletions of chromosome 7 or 5q, and complex karyotypes - worse
features - fatigue, fever, infections, easy bruising, and bleeding, Hepatomegaly and Splenomegaly,
myeloblasts may show various features, including round nuclei, a prominent nucleolus, and a relatively large amount of cytoplasm with azurophilic granules or Auer rods, common im APL
most important lab test - clotting screen (exclude DIC)
Cytogenesis - prognosis & diagnosis
✅ AML = ≥20% myeloid blasts; urgent treatment needed.
✅ Genetic testing guides prognosis & therapy (e.g., FLT3, NPM1).
✅ APL (M3) is a medical emergency – give ATRA immediately!
✅ Allo-SCT is curative for high-risk AML.
83
Iron diefficiency anemia
coilinichia chararacteristic
Chronic blood loss from conditions like peptic ulcers, gastritis, esophagitis, colon / other cancer - 10%, or angiodysplasia
Malabsorption, arthritis, Inadequate Diet
84
Hemophilia A,
genetic bleeding disorder caused by a deficiency of clotting factor VIII, X-linked recessive
prolonged partial thromboplastin time (APTT) test, along with normal prothrombin time (PT) and platelet count, normal bleeding time
85
Hemophilia B, also known as Christmas disease
hereditary bleeding disorder caused by a deficiency of blood clotting factor IX
X-linked Recessive
86
Mantle cell lymphoma (MCL)
a rare, aggressive type of non-Hodgkin lymphoma (NHL) that develops from B-cells, specifically in the mantle zone of lymph nodes, often affecting older adults and men, and can spread to other parts of the body.
type - B-cell lymphoma, t(11;14)(q13;q32)
87
Disseminated intravascular coagulation (DIC)
characterised by activation of coagulation pathways, resulting in formation of intravascular thrombi and depletion of platelets and coagulation factors.
Clinical history can include epistaxis, gingival bleeding, haematuria, oliguria, cough, dyspnoea, fever, delirium, and coma. Physical examination may reveal petechiae, ecchymosis, gangrene, mental disorientation, hypoxia, hypotension, and gastrointestinal bleeding
Diagnosis is based on presence of ≥1 known underlying conditions causing DIC plus abnormal global coagulation tests: decreased platelet count, increased prothrombin time, elevated fibrin-related marker (D-dimer/fibrin degradation products), and decreased fibrinogen level.
Treatment of DIC depends on the underlying condition and presentation, but options include fresh frozen plasma, platelet concentrate, antithrombin III, and heparin.
88
Hairy cell leukemia (HCL)
associated with a high frequency of the BRAF V600E mutation
accumulation of abnormal B cells with "hairy" projections in the blood, bone marrow, and spleen, leading to pancytopenia and splenomegaly.
89
patients on analogues for cancer (fludrabines) need irradiated blood
at the risk of graft vs host disease
(pancytophenia, liver disfunction, dierrhoa, rash)
If on Fludrabin - cotrim should be started to prevent pneumocystis
90
polycythemia,
mutations in hemoglobin can lead to a high oxygen affinity, causing the body to produce more red blood cells to compensate for the reduced oxygen delivery to tissues
91
Myelofibrosis (MF)
a rare blood cancer where scar tissue (fibrosis) builds up in the bone marrow, hindering its ability to produce blood cells, and is part of a group of blood cancers called myeloproliferative neoplasms (MPNs).
can convert to AML
fatigue, shortness of breath, an enlarged spleen (splenomegaly), and in some cases, an enlarged liver (hepatomegaly), along with other symptoms like weight loss, night sweats, and easy bruising or bleeding.
blood picture - teardrop poikilocytes (dacrocytes), along with other features like nucleated red blood cells and immature granulocytes.
diagnostic criteria - major - Megakaryocyte proliferation and atypia, Reticulin and/or collagen fibrosis, not meeting myeloid neoplasms
JAK2V617F or other clonal markers
Minor - 2 of Leukoerythroblastosis/ Increased LDH/ Anemia/ Palpable splenomegaly
92
pneumococcal vaccine before spleenectomy
min 14 days
93
zieve syndrome triad
jaundice, hemolytic anemia, and hyperlipidemia.
treatment - supportive care and complete abstinence from alcohol.
94
burkitt lymphoma
a rare, fast-growing, and aggressive type of non-Hodgkin lymphoma that develops from B-cells, a type of white blood cell, and can affect children and adults.
rapidly enlarging lymph node masses or abdominal tumors, and constitutional symptoms like fever, night sweats, and weight loss
Three main types of Burkitt lymphoma:
Endemic: Primarily occurs in equatorial Africa and is associated with Epstein-Barr virus (EBV) and chronic malaria.
Sporadic: Occurs outside of Africa and is less commonly associated with EBV.
Immunodeficiency-associated: Usually associated with HIV infection or the use of immunosuppressive drugs.
Common regimens include R-CODOX-M/R-IVAC (rituximab, cyclophosphamide, vincristine, doxorubicin, methotrexate, ifosfamide, etoposide, and cytarabine)
95
felty syndrome
a rare complication of rheumatoid arthritis (RA) characterized by the triad of RA, neutropenia (low neutrophil count), and splenomegaly (enlarged spleen).
96
Delayed blood transfusion
97
cryoglobulinemia iii
Type III:
This type is characterized by polyclonal IgM with RF activity, also along with polyclonal IgG,
98
Autoimmune hemolytic anemia (AIHA)
Strongly positive direct antiglobulin test (DAT)
Treatment often involves immunosuppressants like corticosteroids
develop gradually or acutely
99
Delayed Hemolytic Transfusion Reaction (DHTR)
typically within a few days to weeks.
DHTR is a delayed immune response to antigens on transfused red blood cells, usually due to a previous exposure to those antigens (e.g., previous transfusion or pregnancy
A positive DAT
supportive care, such as hydration and transfusion with antigen-negative blood
100
Stomatocytosis
Stomatocytosis is a condition characterized by red blood cells (RBCs) with a distinctive "mouth-like" or "slit-like" central pallor on a blood smear. These cells are also known as stomatocytes. Stomatocytosis can be either hereditary (genetic) or acquired. Hereditary stomatocytosis is a rare, autosomal dominant condition that can cause hemolytic anemia and other complications. Acquired stomatocytosis is less common and can be associated with various conditions like alcoholism, liver disease, or certain medications.
101
Hereditary elliptocytosis (HE)
hereditary ovalocytosis, is an inherited blood disorder characterized by abnormally shaped, oval-shaped red blood cells (RBCs). This condition is caused by mutations in genes that encode for proteins in the red blood cell membrane, such as spectrin, protein 4.1, or glycophorin C. HE can range from asymptomatic to severe hemolytic anemia.
autosomal dominant
102
The investigations demonstrate pancytopenia with blood film appearances consistent with bone marrow infiltration (a leukoerythroblastic film). The other options may all induce pancytopenia but would be associated with different blood film appearances. Aplastic anaemia and hypothyroidism generally are associated with a normal blood film, myelodysplasia with abnormal blood cell appearances (e.g. poikilocytosis) and vitamin B12 deficiency with the characteristic hypersegmented neutrophils. A further clue in the stem is the haematuria, which indicates an underlying pathology
103
bone marrow metastases
characterized as osteolytic, sclerotic (osteoblastic), or mixed on imaging studies
Leukoerythroblastic Blood Picture:
This term refers to the presence of immature white blood cells (leukocytes) and red blood cells (erythrocytes) in the peripheral blood, often accompanied by teardrop-shaped red blood cells, indicating that the bone marrow is under stress and producing blood cells in an abnormal way
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Squamous cell carcinoma (SCC) involvement of the skin
Stage 0: The cancer is only in the top layer of skin (epidermis).
Stage I: The cancer has spread into the top and middle layers of skin (epidermis and dermis).
Stage II: The cancer has spread into the top and middle layers of skin and may involve nerves or deeper layers (epidermis, dermis, and subcutis).
Stage III: The cancer has spread to nearby lymph nodes.
Stage IV: The cancer has spread to other parts of the body, such as the lungs, liver, or brain
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Acute lymphoblastic leukaemia (ALL)
hysto - which are immature white blood cells, often with a high nuclear-to-cytoplasmic ratio and a regular, round nuclear shape. The cytoplasm may appear agranular and weakly basophilic, and nucleoli might be inconspicuous.
✅ Adult ALL is biologically distinct from pediatric ALL (more high-risk genetics).
✅ Pediatric-inspired protocols improve outcomes in younger adults.
✅ TKIs + chemo are standard for Ph+ ALL; allo-SCT for high-risk.
✅ Immunotherapy (blinatumomab, CAR-T) transformed R/R ALL management.
