This presentation gives general overview of Quantitative Platelets disorders

1. Quantitative Platelets Disorders
Iqra Yasin
Resident, Obs & Gyne Unit I
SIMS/Services Hospital, Lahore

2. 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

3. Classification
PlateletsDisorders
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)

4. 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)

5. 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

6. Evaluation of Patient with Thrombocytopenia
• 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

7. Evaluation of Patient with 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

8. 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

9. 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

10. 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

11. Autoimmune Thrombocytopenia (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)

12. Autoimmune Thrombocytopenia (AITP)
• 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)

13. Autoimmune Thrombocytopenia (AITP)
• 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

14. Autoimmune Thrombocytopenia (AITP)
• 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

15. Autoimmune Thrombocytopenia (AITP)
• 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

17. 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

18. 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

19. Clinical Manifestation of Thrombocytosis
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

20. Clinical Manifestation of Thrombocytosis
Pregnancy complications
• Recurrent Spontaneous
abortions
• Placental Infarction
– IUGR
– Fetal death

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

22. 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