Hemato-Oncology Flashcards
What metal poisoning can Porphyria cause ?
Lead through inhibition of ALA in heme synthesis
Basophillic stripping (aggregates of ribosomes or fragments of ribosomal RNA precipitated throughout the cytoplasm of circulating erythrocyte)
(Basophilic stippling occurs because of imperfect RNA resolution and abnormal ribosomal structure)
Causes include :
1.lead poisoning
2.Hemolysis
3.Thalassemia
Lead poison
Basophillia stripping
Blue Burton line on Gum
Demyelination of motor axon
Memory deficit
Arthralgia Myalgia
GI symptoms
How to know duration of lead poison ?
FEP -check intoxication of past 120 days
Serum lead - exposure within 3 weeks
Plummer Vinson syndrome
Atropic glossitis
Esophageal web
Microcytic anemia
CRAB mnemonic
Multiple Myeloma
Elevated Calcium , Renal Failure , Anemia, Bone lesion
MGUS
Monoclonal Gammopathy of undetermined Significance
M protein ( Immunoglobulin ) < 3 g/dl
Asymptomatic
30% May progress to MM
ABVD regime
HL
Adriamycin/Docorubicin
Bleomycin
Vinblastine
Decarbazine
Androgen Excess (Hirtuism+acne+irregular period ) + Adnexal mass +Ovarian Ca ?
Sertoli leydig ovarian tumor
(Sex cord stromal tumor) -Benign
Sertoli cell / Leydig cell of ovary function ?
S- convert Testosterone to estrogen (Spermatogenesis)
L-secrete testosterone
vWF disease
Factor VIII carrier
Platelet adhesion
Deep Bleeding (Joint )
(Platelet disorder )
Adult , Bloody diarrhea
5 cardinal signs
1.Renal Insufficiency ,
2.Anemia
3.Fever ,
4.CNS problems,
5.Thrbocytopenia
TTP/HUS
E.coli toxin»_space; endo cells damage >thrombosis+plt activation
Rx of TTP/HUS
Plasma Exchange
Immunosuppressant
Painless , round,firm,non-illuminating testicular mass ,elevated LDH
Testicular tumor (Seminoma)
Translluminate testicular mass + Cystic fluid collection
Hydrocele,Vericocele
Painful testicular mass+ elevated WBC
Epidydimitis /Orchitis
Most common germ cell tumor of women
Dysgerminoma (ovarian Tumor)
IncreaseD ALP ,LDH
Causes of increased b-HCG
normal pregnancy
Gestational trophoblastic disease ( Choriocarcinoma , Hydatiform mole ,ectopic pregnancy )
Child
Increased MCHC
normocystic anemia(Intravascular Hemolysis )
Small ,Round RBC
H. Spherocytosis
Osmotic fragility Test
Direct Coomb - Neg
Most common lung cancer
NSCLC
1. Adenocarcinoma ( Acinar)
2.squamous cell carcinoma (hilum )
Which lung ca are not related to smoking risk?
Large cell lung cancer
Bronchoalveolar Ca
Risk factors of SCLC
Smoking
Asbestos
Aromatic carbon
Ionizing radiation
What condition (syndrome ) is associated with SCLC ?
Lambert Eaton MG (Paraneoplastic Syndrome )
Ca channel antibodies at pre-syneptic NMJ
Lambert Eaton MG features
Ptosis
Diplopia
Muscle weakness
Cause of Pain Crisis in Anemic patient with Jaundice ?
Sickle Cell Anemia ( parvovirus-19)
Pain crisis is due to Sickle disease and b-thalassemia
What causes Sickle cell anemia ?
Assault to RBC due to hypoxia / dehydration»_space; Vascular congestion»_space;sickle»_space; thrombosis»_space;DIC
Missense mutation of b-Hgb
Child (3yr)
Chalky ,White retinal mass in the central eye
Esotropia
Vision , movement ,reflex intact
Retinoblastoma ( Rb TSG)
-Cong Cataract
-abnormal vitreous /retina
Inflammation
Retinoblastoma secondary malignancies ??
Soft tissue sarcoma
Osteosarcoma
Malignant melanoma
Mets via : SAS, Blood ,lymph
Rx of Retinoblastoma ?
Removal of Optic Nerve to prevent brain metastasis
Increased Hgb
Hct 62%(high)
Plt high
Blur vision , rib pain
Primary polycythemia Vera ( EPO normal or decreased )
EPO level in secondary PCV ?
Increased due to renal stimuli
( Eg : Renal tumor , hypoxia, high altitude , EPO receptor mutation )
Polycythemia can cause what carcinoma?
Renal Cell Ca
HCC
How can VSD or PDA causes Polycythemia Vera ?
LΒ»R shunt first ,later pulmonary Vascular resistance allow RΒ»L shunt»_space; deoxygenated blood»_space; Cyanosis»_space; EPO stimulation»_space; RBC increased
3 types of Myeloproliferative disorders
Increased RBC ( PCV)
Increased WBC ( AML)
Increased Plt ( 1 essential thrombocytosis )
Painless jaundice + Abd pain + RUQ mass
Pancreatic cancer
Whipple procedure
Resection of
Antrum + part of duodenum +head of pancreas + GB
Most common brain tumor in child
Medullablastoma
Cerebellar tumor
Hydrocephalus ( large ,shiny scalp , visible veins,downward gaze ,increase ICP)
Most common brain tumor in adult
Glioblastoma ( Butterfly gliomas)
Cerebral Hemisphere + Corpus callosum
Oligodendroma
Slow growing > transient symptoms
Frontal lobes
Glial cells ( made up myelin of axon )
Parasthesia + weakness
Recent infection
Elevated calcium /Cr
Back pain
M protein
Multiple Myeloma
Bence Jone protein
Multiple myeloma
Punch out lytic bone lesions
Multiple myeloma
Baby
Abdominal mass
Watery diarrhea
Irritable
Neuroblastoma (SNS malignancy)
Neuroblast migration along sympathetic ganglia of adrenal medulla
Hypersegmented Neutrophil
Macrocytic Anemia
B12( Megaloblastic Anemia )
Subacute combined degeneration of dorsal column of Spinal Cord
B12 deficiency
Loss of Vibration + Proprioception
Hemoptysis
Vocal hoarseness
LOW/LOA
SOB
Sputum : Atypical cells
Pancoast Syndrome
Lung cancer ( UL )
Pancoast syndrome
UL lung
Pain/ numbness in ipsilateral arm
Horner syndrome
Recurrent laryngeal n compression
Paraneoplastic syndrome
Neck or facial swelling ( SVC obstruction )
Tired
Abd pain
Joint pain ,muscle ache
Gum tooth blue
Lead poison
Child
Diffuse Purpura
Epitaxis
Large platelets ,Prolonged BT
coomb test :Pos
ITP
Coomb : + because Antibodies to plt
Mechanism of low platelet count ?
- Splenic sequestration
2.Decrease production ( Aplastic Anemia , clopidogrel ,aspirin ,SC failure ,leukemia )
3.increase destruction ( MAHA, TTP,ITP , Heparin,Quinidine)
Cells of Ovarian teratoma
Bone , cartilage ,hair , teeth ,glands
Child (8 month)
Deep bleeding + Briuise over knees + increase aPTT ,normal BT
Hemophilia A - factor VIII
Bleeding Joints/ muscles /CNS
Rx of Hemophillia
Mild - decompression
Severe - Factor VIII recombinant
Non-tender palpable neck or supraclavicular mass
Fever +night sweat+ fatigue
Owl eye
Hodgkin lymphoma
Reed stenberg cells
B cells
CD 15+/30+
High transferrin
Low TIBC
High serum Iron
High ferritin
Hereditary Hemochromatosis
Causes of DIC
Infection including sepsis
Malignancy
Pregnancy complication ( abruption of placenta , amniotic fluid embolism)
Trauma
Snake venom
Guaiac Test
FOBT for CRC and GC
Most common adult blood cancer
CML
mature granulocytes : Neut/ESO/Baso, platelet
Splenomegaly
ABL-BCR gene fusion
AML
Tyrosine kinase activity increase
Avoid apoptosis
Imatinib
TKI used in AML
Abd tenderness ,decreased bowel sound
Abdominal mass involving ileum
Lymphoid cells
Burkit lymphoma ( most common NHL type )
Involved GI ( mostly ileocecal / peritoneum )
Starry Sky cells
Burkitt Lymphoma
Maxillary /mandibular mass
African/endemic
Fever/sweat
Burkitt
EBV /HIV /AIDS associated lymph nodes mass
Burkitt Lymphoma
Flushing
Erythema
GI symptoms ( chronic Watery diarrhea)
Caricinoid syndrome
Arising from Small bowel carcinoid tumor
Breast cancer types
1.Sporadic ( HER2) - cell cycle pathway of Estrogen /progesterone signaling
2.Hereditary (BRCA 1/2)- defect in DNA mismatch repair
HbF increase in
B-thalassemia Major
HbA2 increases in
B-thalassemia minor
Alpha thalassemia major
Deletion of 3 alpha alleles -HbH (or )
all/4 alpha alleles-Hb Bart( Hydrops fatalis )
Alpha thalasssemia minor
Deletion of 2 alleles- trait
Deletion of 1 alleles - silent Carrier
Hypocellluar marrow with fatty infiltrate
Pancytopenia ( infection + bleeding + pallor + purpura)
Aplastic Anemia
Adult (65 yrs)
Auer Rod ( needle like projection )
LOW/LOA , Bleeding , Cellulitis increased Blast
AML
Most common childhood leukemia
ALL
Increased immature white cells
Anemia
Increased LDH
Porphobilinogen deinase deficiency
Elevated ALA synthase , Increase ALA
Acute intermittent porphyria
Transplant rejection types
Acute
Subacute
Chronic
What happen in acute transplant rejection ?
Preformed antibodies to graft organs ( antigen) leads to immediate immune response
Subacute transplant rejection
After 1 week
Donor MHC cells started immune response in host ( T cells recognize and attack graft t/s)
Chronic transplant rejection
Weeks and months
Vascular damage mostly
Complement/ T cells /cytokines infiltrate
Who undergoes HSC transplant or BM transplant are at risk of what condition after procedure ?
GVHD
Donor immune system attacks host cells antigen ( Donor T cells attack host tissue )
Initial Rx for ITP
Corticosteroid
Later IVIG ( raise plt count)
Splenectomy (refractory cases)
Antibody to GpIIb/IIIa
Bleeding disorder
Most common children after viral infection /immunization
ITP
Defect enzyme ADAMTS13
Autoantibody to platelet
Seen in female mostly
TTP
Seen in children with E.coli (undercook beef)
Renal insufficiency
Petechiae
MAHA
HUS
Increase BT
Decrease plt count / increase Megakaryocyte BM
Schistocytes
Normal PT/aPTT
MAHA
(TTP & HUS )
Genetic Gp In deficiency
Plt adhesion impaired
BSS ( Barnard Siulier Syndrome)
Plt decrease
Large plt
Gp IIb/IIIa deficiency (genetic)
Glanzman ( platelet disorder)
Drugs that Irreversibly inactivate COX
Aspirin
Function of thrombin
Stabilize platelet plug by converting fibrinogen to fibrin also called 2 Homeostasis
2 homeostasis disorder
Rebleeding after Sx
Tooth extraction
Coagulation factor defect
Hemophilia
Abnormal platelet agglutination test ( Ricocetin test)
Increase BT , aPTT
Mucosal /skin bleeding
VWF defects
Rx: Desmopressin
FVIII deficiency
X linked
Male
Hemophilia A
Vit K deficiency causes;
Newborn - lack of K+ producing gut flora
Malabsorption / Malnutrition
Long term antibiotic uses
Warfarin overdose Rx :
Vit K IV 10 mg
FFP
4 or 3 Prothrombin complex concentrate
May be tranxenemic acid
Screening test for DIC
D-diner ( usually elevated )
MOA of aminocaproic acid
Blocks activation of plasminogen
Types of embolus
Arthelerosclerotic (cholesterol plague)
Fat (bone fracture /soft tissue injury)
Gas ( decompression sickness / laparoscopic Sx)
Amniontic ( dyspnea/ neurologic symptoms /DIC)
PE presentation
Pleuritic chest pain
Hemoptysis
Dyspnea /SOB
Pleural effusion
V/Q mismatch
Elevated D dimer
Wedge shape infarct
Primary Pycythemia aka
Polycythemia Vera
Polycythemia Vera Dx criteria
JAK - 2 mutation
Erythrocytosis (high RBC/WBC/plt)
Hematocrit In polycythemia Vera
Male >0.50
F > 0.48
JAK -2 negative PV criteria
Red cell mass >25%
Hematocrit high
BM histology consistent with PV
With at least 2 of the following :
Splenomegaly
Low serum EPO
Neut leukocytosis , thrombocytosis