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Newsletter

Sunday, October 22, 2017

Anaemias and Bleeding Disorders

Anaemia: Most common problem in medical clinics the world over. Major public Health importance
Chronic disability
Poor quality of life
Low income




Definition:
The level of circulating Hb or  Red cell is lower than that in healthy subjects of the same sex and age group and in the same environment.

Anaemic patient suffers from tissue hypoxia, the consequence of a low oxygen carrying capacity of the blood.

Clinical manifestations are determined by its aetiology and pathogenesis.

Clinical features of anaemia

Symptoms
May be asymptomatic (Increase in 2 -3 DPG)
Fatigue
Headaches 
Dizziness and faintness
Angina 
Palpitations
Intermittent claudication

Signs
Pallor 
Tachycardia
Cardiac failure 
Papilloedema 
Retinal heamorohages
Angular stomatitis
Koilonychia 
Brittle Nails and hairs 
Atrophy of tongue papillae 
Dysphagia and glossitiis (Plummer – Vinson syndrome )
Chronic leg ulcers
Bone deformities – gnathopathy etc.
Beefy Red, Sore tongue
Polyneuropathy
Sub-acute combined degeneration 
Symmetrical paraesthesia ( fingers & toes) 
Loss of vibration sense
Ataxia
Dementia 
Optic atrophy   

Normal Haematological Values

Nigeria                         U.K.
Retics 25 - 85X109/L 25 - 85X109/L
WBC 3.0 - 9.0                 3.7 – 10.0X109/L
Platelets 100 – 300          130 – 400X109/L
Hb 12 – 17                         13 – 17g%
Hct 0.37 – 50                         0.40 – 0.5 L
MCV 80 – 95                 82 – 102fl
MCH 27 – 32                 27 – 32 pg
MCHC 32 – 35                 31.5 – 35 g%
RDW 10 – 15%         10 – 15%
RBC 3.8 – 5.8                 4.4 – 5.5X1012/L

Laboratory Tests in the Diagnosis of Anaemia

Hct Manual (cv 2%)
Automated
Hb
RBC
Red Cell Indices
MCV
MCH
MCHC
RDW
HDW
% Microcytic
% Hypochromic
Retic Count and Retic Indices
ESR


RED CELL MORHPHOLOGICAL ABNORMALITIES

Macrocytes
Macro ovalocytes
Microcytes
Miccrospherocytes
Ovalocytes (Elliptocytocytes)
Sickle cells (Drepanocyctes)
Schistocytes (RBC Fragments)
Acanthocytes
Hypochromia
Poly chromasia
Basophilic Stippling 
Howell - Jolly bodies ( nuclear remnants)
Heinz bodies
Cabot Rings
Nucleated RBC
Target Cells
Ecchinocytes (Burr Cells)


Other specific tests in anaemias?

Hb electrophoresis PH 8.4 
                               PH 6.8
Sickling Tests
Osmotic fragility
Globin Chain synthesis
DNA analysis
B12
Serum and RBC folate assay
Hams Test for PNH)
Urine Haemosiderin
NAP
Viral Studies (HBV, HCV, HIV, EBV)
Red cell survival studies 
M protein , Igs, Skeletal survey
Bone Marrow culture for TB
Marrow Examination for Leishmaniasis
FNAC/LN Biopsy
Splenic aspirate
Rheumatoid Factor
ANA
Abdominal US and CT Scan
Laparotomy and Splenectomy


Megaloblastic anemia

Hb (g/dL)                    6.2
Hct (%)                      21.3
RBC (x 106/µL)                1.70
MCV (fl)              125.7
RDW (%)                          20.9
MCHC (g/dl)                     34.5
HDW (g/dl)                       4.10
% Macro                 60.4
%Hypo                               3.2
Platelets (x 103/µL)       184
MPV (fl)                             9.7


Megaloblastic anemia Rectic Profile

%Reticulocytes                             2.1
Absolute reticulocytes              
 
(x 109/l)                                      63
%low staining-intensity                68.2
%Medium staining-intensity       24.6
%High staining-intensity             7.2
MCVr (fl)                                    138.5
RDWr (%)                                    14.9
CHCMr (g/dl)                              24.8
HDWr (g/dl)                                  3.1
CHr (pg)                                      33.5
CHDWr (pg)                                5.5   


Iron-Deficiency anemia

Hb (g/dL)                    8.2
Hct (%)                       26.8
RBC (x 106/µL) 3.36
MCV (fL)                    79.9
RDW (%)                      17
MCHC (g/dL)              31.1
HDW (g/dL)                3.89
MCH (pg)                    24.4
% Micro                      6.1
%Hypo                        22.3


Iron-deficiency anemia Retic Profile

% reticulocytes                          2.0
Absolute reticlocytes (x 109/L       98
%low staining-intensity            86.9
%Medium staining-intensity       9.5
%High staining-intensity             3.6
MCVr (fL)                                89.5
RDWr (%)                              22.3
CHWr (g/dL)                          20.3
HDWr (g/dL)                          3.50
CHr (pg)                                17.2
CHDWr (pg)                          3.6


ANAEMIA IN CHRONIC RENAL DISEASE

Acute or chronic Renal failure produces a normochromic-normocytic anaemia
Ecchinocytes (Burr cells) in the blood film
Retic count: normal or low
Bone marrow: normoblastic erythropoiesis without erythoid hyperplasia
Dominant cause of anaemia in Renal failure is reduced level of EPO

ANAEMIA IN CHRONIC RENAL DISEASE  {contd}

*anaemia occurs in 60-80% of patients with renal impairment
Low Hb is an independent risk factor for increased mortality
Causes of anaemia in Renal Disease
Diminished EPO production
Bleeding: Platelet dysfunction, thrombocytopaemia
MAHA
Vit K deficiency (nutritional)
Folate deficiency: associated with haemodialysis
Fe deficiency: associated with haemodialysis
Blood loss at haemodialysis
Neocytolysis (selective destruction of young RBC; can be precipitated by bolus IV EPO


Bleeding Disorders

===> Acquired Bleeding Disorders
===> Inherited Bleeding Disorders

The acquired coagulation disorders

Deficiency of Vit K – dependent factors, 
Haemorrhagic diagnosis of the new born.
      Biliary obstruction
      Malabsorption of Vit K Sprue & gluten-induced enteropathy
      Vitamin k – antagonist therapy if Coumains
Liver Diseases
DIC
Inhibition of coagulation 
      Specified inhibitors – e.g. antibodies to FVIII
      Non specified  - antibodies SLE, Rheum, arthritis
Miscellaneous
      Dxs with M-protein production
      L – Asparaginese
       with heparin, defibrinating agents
      Massive transfusion syndrome.


DIC

Definition: Pathological syndrome resulting from the  formation of thrombin, subsequent activation and consumption of certain coagulant proteins, and production of fibrin thrombin.


Normal Response to tissue damage.

                      Thrombin      ⇒          Coagulation
              ⇗                released                        of blood ⇘
 Tissue
Damage
                                                                               stops blood clot



                                      Thrombotic features
                                                     ↑
   Unregulated        ⇁              Free              ⇀              Widespread
     Thrombin                     Thrombin                        microvascular 
                                                                                      thrombosis
Body attempts vascular                                                        ⇃
Patency
     ⇃                                                                           Tissue Ischaemia
                                                                                           +
Plamin ++     →                   Haemorrhagic                    Organ Damage 
                                        manifestation
    ⇃
Systemic and Local
 fibrinogenolysis                 


Thrombin is naturally contained by antithrombin / Protein C                                     



                              Causes of DIC

 Acute DIC                         Chronic DIC
- Infection                  Malignancy
- Obstetric complications                 Retained Dead fetus
- Trauma                 Liver Disease
- ABO incompatible red cells         Liver Disease
- Liver Dx                 Sever localized
                                                                Intravascular coagulation


INHERITED Bleeding Disorders

Haemophilia A     
B
C
Von Wille brands Sisease
History
Haemostatic challenge
Severity




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