Pathology

Question 1

Defect in ALA synthase

Anemia…
Prussian blue..
Tx…

Answer 1

Sideroblastic anemia
(Defect in ALA synthase, isoniazid etc)
#RINGED SIDEROBLASTS W/ Prussian blue !
Tx: Pyridoxine B6

Question 2

Electronic Microscopy

“Tennis Racket” Intracytoplasmatic granules

Answer 2

Langerhans cells
Birbeck granules
-
Child w/ lytic bone lesions + skin rash or recurrent otitis media.. 
Histiocytosis de Langerhans

Question 3

Palpable purpura
Arthralgias
Abdominal pain
Hematuria

Inmunocomplex IgA deposition

Answer 3

Henoch-Schonlein Purpura

Question 4

PaO2 / SaO2 / O2 contnet

ANEMIA
HIGH ALTITUDE
CYANIDE POISONING
CO POISONING
POLIYCYTHEMIA

Answer 4

ANEMIA n n l
HIGH ALTITUDE l l l 
CYANIDE POISONING  n n n 
CO POISONING n l l
POLYCYTHEMIA n n h

Question 5

Prolonged TP
Normal KPTT
factor

Answer 5

VII

Extrinsic pathway!

Question 6

Many blood transfusions

Bx: Kupffer -> yellowish-brown granules

Answer 6

Hemosiderin

Question 7

Myeloproliferative disorders
mutation
symptoms

Answer 7

CML - t (9:22) -Philadelphia- #basophils/splenomegaly -IMATINIB

Polycythemia vera - JAK2/STAT -erythromelalgia-itching after a hot shower - RUXOLITINIB (JAK1/2 -)

Essential Thrombocythemia - 50% JAK2- bleeding/thrombosis

Myelofibrosis- 50% JAK2 - bone marrow fibrosis- #Splenomegaly #teadrop RBC -

Question 8

mutation RB1 gene

risk

Answer 8

Retinoblastoma

Osteosarcoma

Question 9

Tissue factor into maternal circulation? (Ej Abruptio Placentae)
Complication?

Answer 9

DIC-
Low platelets
increase TP and KPTT
High D-Dimer

Question 10

Old man
Fatigability, Dyspnea
A decrease in low extremities vibratory sensation
Treatment?

Answer 10

Vitamin B12

Question 11

Benign lymph node response Vs. Malignant

Answer 11

Benign-> Policlonal Proliferation

Malignant-> Monoclonal Proliferation

Question 12

Prosthetic heart valve
RBC fragments
Burr cells
Helmet cells

Answer 12

Mechanical trauma

macroangiopatic anemia

Question 13

caucasian
Thrombosis without explanation
Resistant to degradation by Protein C

Answer 13

Factor V Leiden

most common inherited hypercoagulability

Question 14

Elevated WBC
Increase in precursor forms
# Leukocyte alkaline phosphatase score low #

Confirm?

Answer 14

Chronic Myelogenous Leukemia

t (9:22)
BCR-ABL1 fusion gene mRNA

Question 15

Pancytopenia
Aplastic anemia
Bx bone marrow

Answer 15

hypocellular bone marrow with FATTY INFILTRATION!

Question 16

Lab in DIC

Answer 16

#Schistocytes#
-
< Platelets 
< Fibrinogen 
< Coagulation factors
< Protein S,C

> PT / PTT/ BT
d-Dimer

Question 17

MARKERS
- ALL

B-ALL
T-ALL

Answer 17

ALL - TdT+

B-ALL -> CD 10+, CD19+, CD20+

T-ALL-> CD1,2,5,7,8. +

Question 18

A MYELOgenous Leukemia

Answer 18

+ MYELOperoxidase

Auer rods

Question 19

TRIAD:
Thrombocytopenia
microangiopathic hemolytic anemia
Acute Renal injury
\+
Feber
Neurologic Symptoms

LAB?

Answer 19

TTP
#Thrombotic Thrombocytopenic purpura#
- ADAMTS13 (a vWF metalloprotease)

Lab:
< platets
Schistosites
Normal TP/kPTT

Question 20

Black gallbladder pigment stones
unconjugated bilirubin
-

Answer 20

Hemolysis

Question 21

Most common inherited coagulation disorder.

LAB?

Answer 21

Von Willebrand

> Bleeding Time
kPTT

Question 22

Hemophilia
Clinic
LAB
TTO

Answer 22

Hemarthroses, easy bruising, bleeding surgery

> kPTT

Question 23

Treatment of APL t(15;17)

Answer 23

ATRA (Vit A- derivate)

Question 24

Schistocytes
###  < Haptoglobin

> LDH
Bilirrubin

Answer 24

Hemolytic Anemia

Question 25

Sickle Cell Disease | manifestations

Answer 25

1-Hemolysis: < haptoglobin 2-Vaso-occlusive Symptoms: Painfulswelling hands and feet #Dactylitis 3-Infections: encapsulated

Question 26

Enzyme that activates 2,7,9,10,S,C with Vit K Newborns receive supplementation of Vit K.

Answer 26

gamma-glutamyl carboxylase

Question 27

Porphyria Cutanea Tarda Enzyme def Symptoms Exacerbated

Answer 27

Uroporphyrinogen decarboxylase ``` #Blistering cutaneous PHOTOSENSITIVITY and HYPERPIGMENTATION Exacerbated by OH- ```

Question 28

Follicular Lymphoma t ( : ) Overexpression gene

Answer 28

t (14;18) | > antiapoptotic BCL-2

Question 29

``` pancytopenia splenomegaly teardrop RBC ? BM aspiration: BM Bx: ```

Answer 29

MYELOFIBROSIS BM aspiration: "dry tap" BM Bx: fibrosis with a cluster of MEGAKARIOCYTES

Question 30

``` Old house paint/Occupational exposure L EE AA D ``` Enzyme?

Answer 30

Lead Lines (Burton) gingivae or metaphysis Rx Encephalopathy and Eritrocyte BASOPHILIC SPLITTING Abdominal Colic & Anemia Drops worst and foot Enzyme: - ALA Dehydratase!