Platelets disorders

Quantitative Platelets Disorders

Iqra YasinResident, Obs & Gyne Unit I

SIMS/Services Hospital, Lahore

Introduction

• The platelets arise from the fragmentation of the cytoplasm of megakaryocytes in the bone marrow– circulate in blood as disc-shaped anucleate particles

for 7-10 days.

• Platelets are responsible for primary hemostatic function

• Normal Count is 150,000/µl to 400,000/µl

ClassificationPl

atel

ets D

isord

ers

Quantitative

Thrombocytopenia(< 150,000/µl)

Decreased Production

Increased Consumption/Destruction

Splenic Sequestration

Pseudo

Aggregates in specimen (rule out on peripheral

smear)

Dilutional

Thrombocytosis(> 400,000/µl)

Qualitative

Inherited (rare)

Acquired (common)

Thrombocytopenia(Count < 150,000/µl)

Failure of Production Platelet Consumption/Destruction

Splenic Sequestation

Bone Marrow suppression Gestational Thrombocytopenia Liver Diseases

Drug Induced Immune Thrombocytopenia• Primary • Secondary

• SLE• Antiphospholipid

Syndrome• Drug induced(HIT)• Infection(HIV, EBV)

Portal HTN

Aplastic Anemia Non immune Thrombocytopenia• DIC• TTP/HUS• Preeclampsia(HELLP

syndrome)• Acute Fatty Liver of Pregnancy

Hepatic or Portal vein thrombosis

Infection(parvo B19) Spleenomegaly

Bone Marrow Infiltration Myeloprolifrative Disorders

(Non-)Hematological Malignancies Lymphoproliferstive Disorder

Severe Vit B12 and Folate Deficiency

Storage Diseases

Tropical infections (Malaria)

Evaluation of Patient with Thrombocytopenia

• Presence of current/previous bleeding problems– Mucocutaneous bleeding

• Skin lesions• Epitaxis, bleeding Gums, menorrhagia

• Past History– HIV, HBV, HCV, SLE and other systemic problem

• Obstetrical History– Recurrent abortion (Antiphospholipid syndrome)

• Family History– Hematological Problems

• Others– Drug, alcohol, tranfusion


• Examination– Petechiae(<2 mm), Purpura(2-10 mm), Ecchymosis (> 10 mm)– Features of CLD, SLE– Splenomegaly (CLD versus ITP)

• Investigations– CBC ( decreased platelet count)– Peripheral smear

–

Red cell Fragmentation DIC, Microangiopathies

Hypersegmented neutrophils Vit B 12 deficiency

WBC/ RBC abnormalites Underlying bone marrow disease

Normal ITP/Gestational thrombocytopenia


• Specific Investigations

ANA, SLE

Lupus Anticoagulant, anti cardiolipin antibodies Antiphospholipid Syndrome

D-Dimer, coagulation screening DIC

vWF-cleaving protease deficiency (ADAMTS 13 activity) TTP

Serum Urate level Preeclampsia, HELLP syndrome, Acute Fatty Liver of Pregnancy

Gestational Thrombocytopenia• 70 % of cases of thrombocytopenia at delivery

• Exact etiology unknown

• Suggested mechanisms includes– Dilutional– Reduced life span– Increased platelet activation in pregnancy in placental circulation

• Differentiated from ITP– Develop late in pregnancy(3rd trimester)– No Pre-pregnancy history of bleeding with normal Platelet count– Return to normal count within 7 days of delivery

Gestational Thrombocytopenia

• Investigation– Normal peripheral smear and bone marrow

examination with low platelet count

• Management– No treatment required during pre-pregnancy and

antenatal period– During labor and delivery and postnatal care

• If ITP not excluded, treat as case of ITP• Otherwise, no specific treatment required

Autoimmune Thrombocytopenia (AITP)

• 3 % of cases of thrombocytopenia at delivery

• Most common cause of thrombocytopenia in 1st Trimester

• Mechanism– Autoantibodies against Platelet surface glycoproteins leading to destruction in

RES

• Investigation– Normal peripheral smear and bone marrow examination with low platelet count– Normal other specific investigations– Platelets-associated IgG or glycoprotein-associated antibodies are not reliable

diagnostic tools in suspected case of AITP


• Maternal Risks– Count < 20,000/µl(severe thrombocytopenia) at

any gestation and < 50,000/µl at delivery– associated high risk of spontaneous bleeding

antenataly and during delivery

• Fetal Risks– IgG antibodies crosses the placenta leading to

fetal thrombocytopenia(<2 % cases)


• Management– Prepregnancy period

• Optimize the patient and consider for splenectomy• Discuss fetomaternal risk associated with ITP and side

effects of drugs given in ITP during pregnancy

– Antenatal Period• AIMS:

– To treat the maternal symptoms of hemorrhage at any stage of pregnancy

– To achieve a safe platelet count at delivery (> 50,000/µl)


• Antenatal Period– To Whom Treatment should be given?

• If symptoms occur or platelet count <20,000/µl at any gestation and < 50,000/µl at delivery even if asymptomatic

– Treatment options• Steroids (Prednisolone 1mg/kg/day)• IVIG ( 1g/Kg for 1-2 days- Peak response at 4-5 days with total duration of response

3-4 weeks)• Splenectomy in refractory cases in 2nd trimester or occasionally in 3rd trimester during

delivery + prophylactic penicillin• Anti-CD20 monoclonal antibodies (Rituximab) and anti-D Ig in refractory cases as an

alternative slpenectomy• Azathioprine in non-responsive cases• Inform pediatrician and anesthetic about the delivery


• During Labor and delivery

– Platelets should be available if < 50,000/µl but transfused only if bleeding

– Avoid epidural analgesia if count <80,000/µl– C-section no benefit over vaginal delivery– Count >50,000/µl safe for vaginal delivery (BCSH and

ASH guidelines)– Avoid traumatic delivery, fetal scalp electrodes and

fetal skull sampling


• Post-natal Period

– Repair the episiotomy and tear promptly– Send cord sample for platelet count– Follow baby platelet count for few days if initial count is low– If baby is symptomatic or count < 20,000/µl then consider for

IVIG– If bleeding starts give platelet transfusion– Vaccination against pneumoccocus, Hib, Menningoccocus to

the mother– Avoid NASID as Postnatal analgesia

Thrombocytosis

Essential ( Primary)• Essential thrombocytosis (a

form of myeloproliferative disease)

• Other myeloproliferative disorders such as– chronic myelogenous

leukemia, – polycythemia vera,– myelofibrosis

Reactive ( Secondary)

• Inflammation

• Surgery (which leads to an inflammatory state)

• Hyposplenism (decreased breakdown due to decreased function of the spleen)

• Splenectomy

• Asplenia (absence of normal spleen function)

• Iron deficiency anemia or hemorrhage

Clinical Manifestation of Thrombocytosis

Constitutional symptoms • 20-30% of patients

• Weight loss is unusual.

• Other symptoms include sweating, low-grade fever, and pruritus.

Bleeding Symptoms• > 100,000/µl due to

abnormal function of platelets

• GI –main site

• Other sites of bleeding include the skin, eyes, gums, urinary tract


Thrombotic Symptoms• Headache/Migraine• TIA• Visual Disturbance

• Microvascular occlusion of the toes and fingers– digital pain; – gangrene; or – Erythromelalgia (burning pain

and dusky extremity congestion)

• Thrombosis of large veins and arteries

• occlusion of the leg, coronary, and renal arteries.

• Venous thrombosis of the splenic, hepatic, or leg and pelvic veins may develop.

• Pulmonary hypertension


Pregnancy complications• Recurrent Spontaneous

abortions

• Placental Infarction– IUGR– Fetal death

Thrombocytosis

• Diagnosis

– Platelet count > 400,000/µl– Megakaryocytic hyperplasia– Splenomegaly (40-50 % cases)– Thrombotic/Bleeding complication– CRP, fibrinogen, IL-6 (secondary thrombocytosis)

Thrombocytosis

• Management– Prenatal period

• Multidisciplinary approach• If symptomatic, consider plasmapheresis• If thrombosis occur, low dose aspirin/heparin• Hydroxyurea• IFN-alpha (limited experience), antagonize the effect of PDGF

– Labor and Delivery• Maintain vigilance for PPH

Health & Medicine

Platelets disorders