Can any1 explain more this Uw question for me?? - boy123

[ArchivalUser]

Rosy, a 16-year-old girt is brought by her mother because she is having menses for 10-12 days for the past 6 months. She is concerned because her brother has some blood disease and he needed several blood transfusions after a cholecystectomy. The patient is otherwise healthy and denies any smoking or alcohol use. Her vitals are. Temperature: 36.8C(98.3F); BP: 124/78 mm Hg; PR: 89/min; RR: 18/min. There is marked pallor under the mucous membranes. Blood work along with coagulation profile is ordered and results are:

WBC 8,600cmm
Hemoglobin 9.0gmtoL
Hematocrit : 26%,
Platelets 96.000mm3
Bleeding Time 8 min
PT 12.1s
APTT 30s
Giant platelets are seen on the peripheral smear. Looking at the bleeding time the physician asks qualitative platelets tests, which showed

Platelet aggregation studies with ADP : Normal
Platelet aggregation studies with ristocetin : Subnormal
VWF Level :Normal

What is the most probable diagnosis?
A. Thrombotic thrombocytopenic purpura
B. Von Willebrand disease
C. Idiopathic thrombocytopenic purpura
D.GIanzmann's Thrombasthenia
E. Chediak-Higashi Syndrome
F. Bemard-Soufier syndrome

[ArchivalUser]

It is B.
Often VWF is normal.

[ArchivalUser]

how about hemophilia possibility, though the option is not given

[ArchivalUser]

hemophilia will be in a boy and will present most likely with hemartrosis.

[ArchivalUser]

Skip the whole qn concentrate with this part :

Giant platelets are seen on the peripheral smear
Platelet aggregation studies with ADP : Normal
Platelet aggregation studies with ristocetin : Subnormal
VWF Level :Normal

With this data you have 3 diseases to differentiate: choices B,D,F
D. is eliminated because ADP test is Normal
B. is eliminated because VWF is normal dispite ristocetin test is subnormal.


THEREFOR remain with F which is thecorrect answer, WHY?

Bernard soulier has an abnormal ristocetin test and on Peripheral smear have GIANT cells.

I hope I have answered your qn.

[ArchivalUser]

PLATELET IS LESS:: DD

ITP,TTP,BERNALD,DIC,HUS,HIT

ITP :EVRY THING IS normal EXCEPT PLATELET
TTP/HUS:: PTT PROLONGED
DIC: PTT/PT PROLONGED,PLATELET LESS
BERNALD:Gp1a DEFICIECY ),ONLY PLATELET IS INCREASE WITH RISTOCETIN ABNORMAL

PLATELET IS NORMALD

vWD,GLANZMANNS

vWDTT INCREASED ,ABNORMAL RISTOCETIN
GLANZ:: ADP ABNORMAL,CLOT RETRACTION INCREASE

FEED BACK

[ArchivalUser]

In Glanzm - platelets do not aggregate in response to ADP
This pt has N ADP test.

Correct if wrong

[ArchivalUser]

ADP TEST NORML SO IT IS NOT GLANZ..

[ArchivalUser]

Answer is F as I tried to do it step wise. Seems still we havent come to an agreement.

Using these platelet aggreation test we differenciate 3 disorders.
Glanzman: DO ADP test results :abnormal to diagnose this disease.

To differentiate VWD and Bernard solier Do two test: 1st Risectin 2nd VWF level
1st test : Both VWD and B.S are abnormal
2nd test: If the test says VWF is normal: dx Bernard solier.