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A 44 –year- old male presented with Purpura and Melaena

Dr. Tanvir Ahmed Mahadi

MD Resident (Phase-A)

Department of Haematology

BSMMU

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Particulars of the patient

  • Name: MD. Rakibuzzaman
  • Age: 44 years
  • Sex: Male
  • Religion: Islam
  • Occupation: Marketing Manager
  • Marital status: Married
  • Address: Khilkhet, Dhaka
  • Date of Admission: 1st April, 2021
  • Date of Examination: 3rd April,2021

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Chief Complaints

  • Multiple rash all over the body for 15days

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  • Passage of black tarry stool for same duration

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H/O present illness:

According to the statement of the patient, he was reasonably well 15 days back.

  • Then he noticed a few rash in his limbs, which gradually increases in number involving both limbs and trunk and the other parts of the body. These were discrete, initially red, then became bluish and black after few days. The lesions are painless, non-itchy, variable in size and shape.

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Continued…

  • He is also complaining of passage of multiple episode of black tarry stool, which is semi-solid in consistency, foul in odour and sticky, not associated with vomiting of blood, Jaundice or abdominal pain. There is no other bleeding manifestation.

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Continued…

  • There is no history of fever, sore throat, joint pain ,weight loss, abdominal pain or mouth ulcer.

With these complaints, he visited a physician and later admitted in our department for better management.

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History of past illness:

  • He had similar episodes of same illness for 3 times in last 4 months and for that he had to admit in hospital for 3 times and treated with on and off steroid and several units of platelet concentrate and red cell concentrate.

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  • No previous history of radiation therapy, any surgery or other significant chronic illness.

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�

Family history :

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He is married. There is no history of any bleeding disorder in the family.

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Personal History:

No history of betel nut chewing, smoking or taking alcohol.

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Drug history

There is no history of intake of any drug related to his symptoms. He was taking on and off steroids for this disease

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Immunization history:

He is immunized according to EPI schedule. He gave no adult immunization history.

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Socioeconomic condition:

He belongs to a middle class family. Monthly income approximately 25 thousand taka.

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Physical examination

General examination:

  • The patient is moderately anemic.
  • There are multiple purpuric spots on different parts of the body including limbs and trunks of varying sizes, which are painless, do not blanch on pressure
  • No jaundice, cyanosis, clubbing, koilonychia, leukonychia or edema
  • No lymphadenopathy or thyromegaly
  • No sternal tenderness

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Continued:

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  • JVP: Not raised
  • Temp: 98.4F
  • Pulse: 112/min
  • BP: 105/65 mm Hg.
  • Resp. rate: 18 br/min

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Systemic examination:

Gastrointestinal system

  • Lips and oral cavity—There are some bleeding spots over the palate
  • Tongue—Normal

Abdomen

  • Inspection: No abnormality
  • Palpation:

Liver—not palpable

Spleen—not enlarged

Kidneys—not ballotable

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Continued:

  • Percussion: No evidence of ascites (no fluid thrill, no shifting dullness).

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  • Auscultation: No abnormality detected.

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  • Other Systemic examination:

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Cardio-Vascular system

Respiratory system NAD

Nervous system

Musculoskeletal system

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What will be the diagnosis ?

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Differential diagnosis

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1. Immune Thrombocytopenia

2. Aplastic Anaemia

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3. Acute Leukaemia

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Investigations:�

  • CBC:

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28/12/2020

16/03/21

22/04/21

Hb (g/dl)

8.1

5.2

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9.8

ESR (mm 1st hr)

25

28

25

Platelet(/cumm *109 )

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23

18

16

MPV

11.8

-

12

T. RBC count (mill/cumm)

3.95

1.84

3.94

MCV (fl)

68

87

85

MCH (pg)

20

25

24.9

MCHC (g/dl)

30

29.4

29.3

T. WBC count (/cumm*109 )

4.4

8

8.39

N(%)

72

86

83

L(%)

20

5

10

M(%)

04

02

5

E(%)

04

02

2

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  • Blood Film (PBF) : 24/01/21

RBC : Dimorphic Blood picture

WBC : Mature

Platelet : Reduced, large platelets.

Comment: Dimorphic anaemia with Neutrophilic leukocytosis

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  • Bone Marrow examination:28/01/21
  • Hypercellular marrow with relatively normal M:E ratio
  • Erythropoiesis is hyperactive with micronormoblastic

and normoblastic.

  • Granulopoiesis is also hyperactive and maturing into segmented form. Myeloid are increased.
  • Megakaryocytes are normal and show some degree of dysplastic change

Comment: Dimorphic erythroid and myeloid hyperplasia

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Date

20/3/21

Anti CMV IgG & IgM

Negative

HBsAg

Negative

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Anti HCV

Negative

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Anti HIV (1+2)

Negative

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Anti DsDNA

Negative

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ANA

Negative

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C3

1.13 g/l

C4

0.369 g/l

Ant H. Pylori IgG

Negative

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INR

1.26

PT

15

APTT

30

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Inv. Contd..

Date

25/3/21

USG of W/A

NAD

Stool for OBT

Negative

Upper GIT Endoscopy

Hemorrhagic gastritis

Colonoscopy

NAD

S. creatinine

1.02 mg/dl

S.electrolytes

Within normal limit

SGPT

75 U/L

RBS

7.7 mmol/L

Urine R/M/E

Normal

Reticulocyte

3.18%

Blood Group

B (+ve)

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Topic discussion on Immune Thrombocytopenia (ITP)�

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  • ITP has previously been called idiopathic thrombocytopenic purpura, immune thrombocytopenic purpura or autoimmune thrombocytopenic purpura (AITP).
  • These terms have been replaced by immune thrombocytopenia.
  • Primary ITP defined as platelet count of less than 100 000/μL in the absence of other causes or disorders that may be associated with thrombocytopenia.

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Definition of ITP

  • ITP is immune-mediated with involvement of autoantibodies, most often directed against the platelet membrane glycoprotein IIb/IIIa, which sensitize the platelet, resulting in premature removal from the circulation by cells of the reticulo-endothelial system.

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Classification of ITP

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Terminology in Disease Duration

Disease Duration Definition

Newly Diagnosed

<3 months

Persistent

3 to 12 months

Chronic

>12 months

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Clinical presentation:

  • Insidious onset.
  • Bleeding after trauma without spontaneous hg with platelet counts >30 000/cmm.
  • Spontaneous hg with counts between 10 000 and 30 000 (ecchymoses and petechiae).
  • Patients with platelet counts <10 000/cmm are at risk for serious morbidity and mortality, although mortality rate is actually quite low.

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Diagnosis:

  • Clinical history & physical examination
  • Complete blood count
    • to rule out other causes of thrombocytopenia
    • to evaluate for secondary causes of ITP
  • Peripheral blood smear
    • reduced numbers of platelets with no other abnormalities (e.g., schistocytes and dysplastic changes)
    • some patients have large platelets (not pathognomonic)
  • There is no diagnostic test for ITP
  • Antiplatelet antibodies are detected in only 50 to 60% of patients
  • Bone marrow study- (Indication)
    • Atypical clinical/ laboratory feature
    • Patient not responding/ relapse to 1st line therapy
    • Age >60 y
    • Before considering splenectomy

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Treatment Plan according to ASH guideline Nov,2019:

  • First-Line Therapies
  • In adults with newly diagnosed ITP, ASH guideline panel recommends against prolonged course (>6 wks) of prednisone in favor of short course (6 wks)

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  • Suggests either prednisone (0.5 - 2.0mg/kg/day) or dexamethasone (40 mg/day for 4 days).

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  • ASH guideline recommend corticosteroids alone rather than rituximab and corticosteroids for initial therapy.

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  • Second-Line Therapies

Adults with ITP lasting ≥3 months who are corticosteroid-dependent or have no response to corticosteroids(ASH guideline).

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  • Thrombopoietin receptor agonist (eltrombopag or romiplostim)
  • Rituximab
  • Splenectomy

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Response:

  • Dependent: require prednisolone >5 mg/day to maintain PLT >30,000/cmm
  • Durable: PLT >30,000/cmm & doubling from baseline at 6 months

Early- at 1 week

Initial- at 1 month

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Refractory ITP Definition

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  • Whose platelet counts do not respond to ≥2 treatments

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  • No single medication to which they respond

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  • Their platelet counts are very low and accompanied by bleeding

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(Review article : First Published: 13TH February, 2020 on American Scociety of Haematology. Authors: Oriana Miltiadous, Ming Hou and James B.Bussel)

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