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28 April 2021
CLINICAL CASE
Haematology
Pamudith Karunaratne
Medical Undergraduate
Faculty of Medical Sciences
University of Sri Jayewardenepura
INTRODUCTION
Miss. xxxxxx xxxxxxx, a 16 year
old school girl from Dehiwala
presented to the haematology
clinic at CSTH for a routine
clinic visit.
2
INTRODUCTION
5 years
back
Age 11
5 months
back
Age 16
Today
Apperently well
Purpuric
rash
B/L LL Oedema
3
From : Dehiwala
Occupation : Student
GENDER: Female
AGE: 16
PATIENT MEDICAL
HISTORY
Defaulted
Treatments
5 years back
Age 11
4
PATIENT MEDICAL
HISTORY
Purpuric rash
● Black coloured patches
● Over the body, Upper & Lower limbs
● 2cm X 3cm Oval shaped
● After 1 Day turned inro RED
● Disappeared in next day
● Alternatively begins in another site
Haematology
clinic LRH
 ↓ PLT
 FBC
PATIENT MEDICAL
HISTORY
Purpuric rash
● Black coloured patches
● Over the body, Upper & Lower limbs
● 2cm X 3cm Oval shaped
● After 1 Day turned inro RED
● Disappeared in next day
● Alternatively begins in another site
5
Other than that, NO
● Other bleeding manifestations like gum bleeding, melena,
haematemesis, PV bleeding (Not attained menarche)
● Fever, Arthralgia, Myalgia, Retro-orbital pain suggestive of
viral fever
● LOA, LOW suggestive of haematological malignancy
● Butterfly rash, Alopecia, Arthritis, Renal/CNS involvement
suggestive of SLE
● history of blood transfusion
● Sepsis, trauma, Snake bite, prolonged bed ridden
suggestive of DIC
● Jaundice, ascites, haematemesis like features of liver
diseases that can lead to hypersplenism
● other drugs or medical conditions
5 years back
Age 11
PATIENT MEDICAL
HISTORY
Purpuric rash
● Black coloured patches
● Over the body, Upper & Lower limbs
● 2cm X 3cm Oval shaped
● After 1 Day turned inro RED
● Disappeared in next day
● Alternatively begins in another site
6
In the LRH,
● She was admitted
● Several blood investigations done
● ANA was negative
● Diagnosed to have ITP
● Treated with Oral Prednisolone 1mg/kg daily, then
reduced the dose over several weeks
After 1 month
● Prednisolone omitted and Dapsone 50mg bd & Folic acid
1mg is given.
● Frequency and the severity of the rash reduced
significantly
● These drugs taken for 2 years
● Then she developed an Urticarial rash so the clinic
decided it as allergy to Dapsone and omitted it.
● Then the clinic visits defaulted & not had relapses
5 years back
Age 11
PATIENT MEDICAL
HISTORY
Came to CSTH with relapse of the rash and some
symptoms of SLE
● BL / LL Swelling up to knee level
● Gum bleeding
● Achy joints and Difficulty in walking
● Fatigue
● Hair loss
SLE is suspected & referred to Rheumatology
Clinic,
● PLT 68,000
● ESR 85 CRP 8.5
● ANA Positive
● Anti ds-DNA positive
7
● 2D echo – normal with EF > 60%
● DRVVT - Dilute Russell's viper venom time - lupus anticoagulant weakly +
● Cervical Lymphadenopathy – LN Biopsy - Normal
● BM trephine Biopsy – suggestive of peripheral disorder of PLT
● Dental Referral done – Chlorhexidine Mouthwash &
Tranexamic acid mouthwash given
5 months back
Age 16
Symptoms
PATIENT MEDICAL
HISTORY PMHx – No Liver diseases, renal diseases, Thyroid
diseases
PSHx – Hx of blood transfusion, Splenectomy not
done
Allergies/Drugs – Mentioned above, Not on
anticoagulants or antiplatelets
Family Hx – No Family Hx of Haemophilia,
bleeding disorders, Renal & liver disease, SLE,
Haematological Malignancies
Social Hx – School girl at Grade 11
Have GCE OLs this year
Family support good
family income is adequate
Nearest hospital is CSTH 8
DIAGNOSIS
SUMMARY ∆ SLE + ITP
Current status of the patient,
Pt is Symptomatically better.
Compliance & clinic follow up is good.
Ix –
PLT 140,000
HGB 12.8
WBC 8.0
Rx
Azathioprine 50 mg mane
Hydroxychloroquine 200mg nocte
Prednisolone 5mg mane
• Corticosteroids
• IVIG
• IV anti-D
(component of
IVIG)
• Immunosuppress
10
Treatment options
For ITP
• Splenectomy
• H. pylori
eradication
ITP has no cure, and relapses may occur years after seemingly successful
medical or surgical management. Most children with acute ITP do not require
treatment, and the condition resolves spontaneously.
11
Immune thrombocytopenia
(ITP) is a syndrome in which
platelets become coated with
autoantibodies to platelet
membrane antigens, resulting
in splenic sequestration and
phagocytosis by mononuclear
macrophages.
DISCUSSION
The resulting shortened life span
of platelets in the circulation,
together with incomplete
compensation by increased
platelet production by bone
marrow megakaryocytes, results
in a decreased number of
circulating platelets
Peripheral blood smear from a
patient with immune
thrombocytopenia (ITP) shows a
decreased number of platelets, a
normal-appearing neutrophil, and
normal-appearing erythrocytes.
ITP is diagnosed by excluding
other diseases
Impaired production
12
↑ Consumption Other
↑ Destruction
CAUSES
For Bleeding
 Leukaemia
 BM Failure
Aplastic Anaemia
Myelofibrosis
Multiple myeloma
Myelodysplasia
Tumour infiltration
 Megaloblastic Anaemia
 Cytotoxic drugs
 Radiation
 ITP
 2ry immune
SLE
Viral – HIV, Dengue
Drugs
 Post transfusion thrombocytopaenia
 DIC
 Thrombotic thrombocytopenic Purpura (TTP)
 Hypersplenism - Dilution
 Platelet functional disorder
 Massive transfusion - Dilution
13
THANKS
Does anyone have any questions?
Pamudith@dr.com
This is where you give credit to the ones who are part of this project.
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14
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ITP and SLE Clinical case presentation by Pamudith Karunaratne

  • 1. 28 April 2021 CLINICAL CASE Haematology Pamudith Karunaratne Medical Undergraduate Faculty of Medical Sciences University of Sri Jayewardenepura
  • 2. INTRODUCTION Miss. xxxxxx xxxxxxx, a 16 year old school girl from Dehiwala presented to the haematology clinic at CSTH for a routine clinic visit. 2 INTRODUCTION
  • 3. 5 years back Age 11 5 months back Age 16 Today Apperently well Purpuric rash B/L LL Oedema 3 From : Dehiwala Occupation : Student GENDER: Female AGE: 16 PATIENT MEDICAL HISTORY Defaulted Treatments
  • 4. 5 years back Age 11 4 PATIENT MEDICAL HISTORY Purpuric rash ● Black coloured patches ● Over the body, Upper & Lower limbs ● 2cm X 3cm Oval shaped ● After 1 Day turned inro RED ● Disappeared in next day ● Alternatively begins in another site Haematology clinic LRH  ↓ PLT  FBC
  • 5. PATIENT MEDICAL HISTORY Purpuric rash ● Black coloured patches ● Over the body, Upper & Lower limbs ● 2cm X 3cm Oval shaped ● After 1 Day turned inro RED ● Disappeared in next day ● Alternatively begins in another site 5 Other than that, NO ● Other bleeding manifestations like gum bleeding, melena, haematemesis, PV bleeding (Not attained menarche) ● Fever, Arthralgia, Myalgia, Retro-orbital pain suggestive of viral fever ● LOA, LOW suggestive of haematological malignancy ● Butterfly rash, Alopecia, Arthritis, Renal/CNS involvement suggestive of SLE ● history of blood transfusion ● Sepsis, trauma, Snake bite, prolonged bed ridden suggestive of DIC ● Jaundice, ascites, haematemesis like features of liver diseases that can lead to hypersplenism ● other drugs or medical conditions 5 years back Age 11
  • 6. PATIENT MEDICAL HISTORY Purpuric rash ● Black coloured patches ● Over the body, Upper & Lower limbs ● 2cm X 3cm Oval shaped ● After 1 Day turned inro RED ● Disappeared in next day ● Alternatively begins in another site 6 In the LRH, ● She was admitted ● Several blood investigations done ● ANA was negative ● Diagnosed to have ITP ● Treated with Oral Prednisolone 1mg/kg daily, then reduced the dose over several weeks After 1 month ● Prednisolone omitted and Dapsone 50mg bd & Folic acid 1mg is given. ● Frequency and the severity of the rash reduced significantly ● These drugs taken for 2 years ● Then she developed an Urticarial rash so the clinic decided it as allergy to Dapsone and omitted it. ● Then the clinic visits defaulted & not had relapses 5 years back Age 11
  • 7. PATIENT MEDICAL HISTORY Came to CSTH with relapse of the rash and some symptoms of SLE ● BL / LL Swelling up to knee level ● Gum bleeding ● Achy joints and Difficulty in walking ● Fatigue ● Hair loss SLE is suspected & referred to Rheumatology Clinic, ● PLT 68,000 ● ESR 85 CRP 8.5 ● ANA Positive ● Anti ds-DNA positive 7 ● 2D echo – normal with EF > 60% ● DRVVT - Dilute Russell's viper venom time - lupus anticoagulant weakly + ● Cervical Lymphadenopathy – LN Biopsy - Normal ● BM trephine Biopsy – suggestive of peripheral disorder of PLT ● Dental Referral done – Chlorhexidine Mouthwash & Tranexamic acid mouthwash given 5 months back Age 16 Symptoms
  • 8. PATIENT MEDICAL HISTORY PMHx – No Liver diseases, renal diseases, Thyroid diseases PSHx – Hx of blood transfusion, Splenectomy not done Allergies/Drugs – Mentioned above, Not on anticoagulants or antiplatelets Family Hx – No Family Hx of Haemophilia, bleeding disorders, Renal & liver disease, SLE, Haematological Malignancies Social Hx – School girl at Grade 11 Have GCE OLs this year Family support good family income is adequate Nearest hospital is CSTH 8
  • 9. DIAGNOSIS SUMMARY ∆ SLE + ITP Current status of the patient, Pt is Symptomatically better. Compliance & clinic follow up is good. Ix – PLT 140,000 HGB 12.8 WBC 8.0 Rx Azathioprine 50 mg mane Hydroxychloroquine 200mg nocte Prednisolone 5mg mane
  • 10. • Corticosteroids • IVIG • IV anti-D (component of IVIG) • Immunosuppress 10 Treatment options For ITP • Splenectomy • H. pylori eradication ITP has no cure, and relapses may occur years after seemingly successful medical or surgical management. Most children with acute ITP do not require treatment, and the condition resolves spontaneously.
  • 11. 11 Immune thrombocytopenia (ITP) is a syndrome in which platelets become coated with autoantibodies to platelet membrane antigens, resulting in splenic sequestration and phagocytosis by mononuclear macrophages. DISCUSSION The resulting shortened life span of platelets in the circulation, together with incomplete compensation by increased platelet production by bone marrow megakaryocytes, results in a decreased number of circulating platelets Peripheral blood smear from a patient with immune thrombocytopenia (ITP) shows a decreased number of platelets, a normal-appearing neutrophil, and normal-appearing erythrocytes. ITP is diagnosed by excluding other diseases
  • 12. Impaired production 12 ↑ Consumption Other ↑ Destruction CAUSES For Bleeding  Leukaemia  BM Failure Aplastic Anaemia Myelofibrosis Multiple myeloma Myelodysplasia Tumour infiltration  Megaloblastic Anaemia  Cytotoxic drugs  Radiation  ITP  2ry immune SLE Viral – HIV, Dengue Drugs  Post transfusion thrombocytopaenia  DIC  Thrombotic thrombocytopenic Purpura (TTP)  Hypersplenism - Dilution  Platelet functional disorder  Massive transfusion - Dilution
  • 13. 13 THANKS Does anyone have any questions? Pamudith@dr.com
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