Quantitative and Qualitative Platelet Disorders

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Questions and Answers

Which of the following is NOT a characteristic of typical HUS?

Shiga toxin enterohemorrhagic Escherichia coli (O157:H7)

Hemolytic anemia

Bloody diarrhea

High white blood cell count (correct)

Which of the following is a common cause of thrombocytopenia in children aged 6 months to 4 years?

Shigella dysenteriae (correct)

Immunosuppressants

Underlying conditions

Chemotherapeutic drugs

What is the prevalence rate of the disorder described in the text?

1 case per 100,000 people

10 cases per 1 million people (correct)

10 cases per 10 million people

1 case per 1 million people

Which condition is characterized by thrombocytosis not exceeding 800,000/uL?

Reactive thrombocytosis Signup and view all the answers

Which population group is more commonly affected by this disorder according to the text?

Females in general Signup and view all the answers

Which of the following is NOT a typical indication for a bone marrow aspiration in a patient with suspected ITP?

Positive Coombs' test Signup and view all the answers

What is the incidence of ITP?

1-3 per 100,000 cases annually Signup and view all the answers

Which of the following is the most dangerous clinical feature of ITP?

Intracranial hemorrhage Signup and view all the answers

What is the typical platelet count range in ITP patients?

30,000 - 80,000 / uL Signup and view all the answers

Which of the following is NOT a typical clinical symptom of ITP?

Fever Signup and view all the answers

According to the American Society of Hematology guidelines, which of the following children with ITP can be managed with observation alone?

Children with no bleeding or mild bleeding (skin manifestations only) Signup and view all the answers

Which of the following best describes the 3As in Primary Hemostasis?

Adhesion, Activation, Aggregation Signup and view all the answers

Which two disorders associated with Albinism can be potentially lethal due to causing hemorrhagic episodes?

Hermansky-Pudlak Syndrome & Chediak-Higashi Syndrome Signup and view all the answers

What is the characteristic platelet defect associated with Glanzmann's thrombasthenia?

Giant pale platelets Signup and view all the answers

In the scenario of Yue post-appendectomy, which pain reliever is most appropriate for pain management?

Paracetamol Signup and view all the answers

Based on Katara's symptoms and lab results, what primary impression can be made about her condition?

Hermansky-Pudlak Syndrome Signup and view all the answers

What is the characteristic feature seen in Bernard-Soulier syndrome?

Prolonged Bleeding Time Signup and view all the answers

What is the primary finding indicated in the laboratory results?

Low platelet count (thrombocytopenia) Signup and view all the answers

What is the main characteristic of the platelets observed in the peripheral blood smear (PBS)?

Giant and pale gray-colored platelets Signup and view all the answers

Which class of medications is commonly associated with drug-induced platelet defects?

Pain killers Signup and view all the answers

What is the primary function of anticoagulants?

Inhibit clot formation Signup and view all the answers

Which class of medications is used in the treatment of endothelial damage, such as stroke or ischemic attacks?

Antiplatelets Signup and view all the answers

What is the most aggressive class of medications mentioned in the text, and under what condition are they used?

Thrombolytics, used within the golden period of a thrombotic event Signup and view all the answers

In Glanzmann's Thrombasthenia, what is the primary defect?

Defect in the GP IIb/IIIa complex, impairing fibrinogen binding to platelets Signup and view all the answers

Which laboratory finding is NOT characteristic of Glanzmann's Thrombasthenia?

Presence of giant platelets on peripheral blood smear Signup and view all the answers

Which of the following is NOT a typical clinical feature of Glanzmann's Thrombasthenia?

Hemophilia-like joint bleeding Signup and view all the answers

What is the diagnostic hallmark of Glanzmann's Thrombasthenia?

Impaired platelet aggregation with all physiological agonists except ristocetin Signup and view all the answers

Which genetic mutation is commonly associated with Glanzmann's Thrombasthenia?

Mutations in the $ITGA2B$ and $ITGB3$ genes on chromosome 17 Signup and view all the answers

In Glanzmann's Thrombasthenia, how does the severity of bleeding typically change with age?

The severity of bleeding decreases with age Signup and view all the answers

Study Notes

ITP Diagnosis

Diagnosis of exclusion: no family history of hemorrhagic abnormalities, no secondary causes
Physical examination: unremarkable except for signs of bleeding
Laboratory parameters: CBC, bleeding time
Bone Marrow aspiration test always warranted, especially for:
- Atypical clinical symptoms
- Age > 60 years old to rule out MDS
- Pediatric age group to rule out Leukemias
- Refractory ITP

Clinical Manifestations

Petechiae
Hematuria
Epistaxis
Noncutaneous bleeding (3-4%)
GI bleeding
Retinal hemorrhages
Mucous membrane bleeding
Severe ITP: hemorrhage (25-50%)

Incidence and Disease Course

Peak incidence: 20-50 years of age
1-3 cases per 100,000 annually
Female > Male
Insidious onset

Laboratory Findings

CBC: platelet count 30,000-80,000/μL
Anemia: extent of blood loss
Bone Marrow aspiration: megakaryocytic hyperplasia
Coomb’s Test: positive for rule in EVAN’S SYNDROME

American Society of Hematology Guidelines on ITP

Pediatric diagnosis and treatment:
- No bone marrow aspiration biopsy necessary for children and adolescents with typical ITP features
- IVIG therapy and corticosteroids may be used
- Observation alone for children with no bleeding or mild bleeding (skin manifestations only)

Primary Hemostasis

3As: Adhesion, Activation, Aggregation

Disorders with Hemorrhagic Episodes

Hermansky-pudlak syndrome
Chediak-Higashi syndrome

Thrombasthenia and Bernard-Soulier Syndrome

Thrombasthenia: deficiency of Gp IIb/IIIa complex
Bernard-Soulier syndrome: deficiency of Gp Ib/IX/V complex

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Description

This quiz covers the diagnosis of Idiopathic Thrombocytopenic Purpura (ITP) as a diagnosis of exclusion, focusing on the history taking, physical examination, and laboratory parameters required for accurate diagnosis. It also discusses the indications for Bone Marrow Aspiration (BMA) test in cases of atypical symptoms, specific age groups, and refractory ITP.