59

DIC
It represent the consumption of coagulation factors due to activation of
coagulation process either by intrinsic or extrinsic pathways with
resultant fibrinolysis.
It may lead to he or thombosis.
C/P Cause. Hge, cerebral Hge, thrombosis elsewhere.

Types
Acute DIC Chronic DIC
Causes  Spesis Cause  Malig i.e. Trousseau’s
 Amm. Fluid E s  Retained dead fetus
 Abruptio placenta
 Acute leuk M3
 ABO in compatibilities
Diag.  Plat. C. marked  Diag.  P.T & P.T.T. may be
  P.T., P.T.T normal, plat mild 
  V, VIII   FDPs
  FDPs  fibrinogen may be
 fibrinogen  normal.
 TT   TT may be normal
ttt off DIC
ㄱ Cause
ㄱ Hge Fr. Fr plasma – fresh blood, plat concentrates,
but theoretically we add fuel to the fire, but
practically this does not occur
ㄱ Thrombosis  heparin
ㄱ EA CA can be used but after heparin to avoid
thrombosis.
N.B.
 They are vix defect they inactive (V, VIII) so they me .
 Throbomdulla from endothelumi – thrombm leading to
activation of protein C.
 60

Aplastic Anaemia

1. Primary idiopathic acquired

An autoimmune mechanism may be responsible.
C/P
 Absence of production of :-
* RBCs  anaemia
* WBCs  infections
* Plat  hge
 Bleeding in skin and m.m., haematuria and epistaxis are
common. Intracranial bleeding is always a risk.
 Necrotic mouth and throat ulcers and monilial infections
reflects the neutropenia.
Investing
 Causes of aplastic an must be excluded.
 History of viral illness particularly hepatitis may be important.
 Blood  pancytopenia.
 B.M.  pancytopenia.
ttt
 Blood transfusion
 Androgen, steroid
 Cyclosporin, antilymphocyte Ab
 B.M. transplantation.

2. Secondary aplastic An
ㄱ It is important to investigate the reported side effects of all
drugs taken over the preceding months.
ㄱ In some instances the cytopenia is more selective and affects
only one cell line most often the neutrophils.
ㄱ The clinical features and methods of diagnosis are the same
as for primary idiopathic aplastic An.
ㄱ An underlying cause should be treated or removed.
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N.B. Causes of Acquired aplastic An.

Drugs * Cytotoxic drugs.
* antibiotic e.g. chloramphenicol
* AutiRh e.g. penicillamine, gold, phenyl butazone,
indomethacin.
* Antithyroids
* Anticonvulsants.
Chemicals * benzene
* Organophosphates
Radiations
Viral hepatttis
P.N.H

Agranulocytosis
It means decrease in the count of granulocytes mainly neutrophils.
Aet * the same cause of 2ry B.M. aplasia

C/P * Recurrent infections

* Sore throat with scanty pus
* The signs of Inflammation are minimal

Investingation
* Bl. Picture  P.N.L. (leucopenia with relative
lymphocytosis)
* RBCs, plat  normal

ttt * ttt of infections

* Ab e.g. penicillins.
* ttt of the cause
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Hypersplenism
It is increase phagocytic activity of the spleen to phagoctyose the blood
elements.
Aet. * 1 ry
* 2ry e.g. portal H-chr. H. An

C/P * Mono or pancytopenia.

* RBCs with chromium phagocytosed inside the spleen.
Invetig * Empty blood + full B.M
ttt * Blood – cause - splenectomy

Hypercoagulable states

ㄱ Medical conditions
1- Malignancy
1. Behcet’s syndrome
2. Nephritic $.
ㄱ Haeatological disorders
1- Polycythaemia rubra vera
2- Essential thrombocytopenia.
3- Myelofibrosis
4- Paroxysmal nocturnal haemoglobinuria.
ㄱ Deficiency of anticoagulants
1- Antithrombin III
2- Protein C
3- Protein S
ㄱ Antiphospholipid antibody
1- Lupus anticoagulant
2- Anticardiolipin
 63

C/P of Hypercoagulable state

ㄱ Manifestation of the cause.
ㄱ Recurrent thrombosis
ㄱ Recurrent DVT
ㄱ Recurrent cerebral thrombosis.
ㄱ Budd chiari syndrome
Investigation and ttt
The investigation and treatment are directed to DVT, cerebral
thrombosis for example and also directed to the cause.

Antiphospholipid antibody
syndrome
In the antiphospholipid antibody syndrome an antibody in the
patient’s plasma has activity against enzymic reactions in the coagulation
cascade.
The antibody, in vitro prolong the P.T.T. as it interacts with the
phospholipid in the reaction tube and inhibits the enzymic interactions of
coagulation components.
When the Ab inhibits coagulation in these ways it is known as the
lupus anticoag.
In some individuals the antibody can be detected because it binds
to cardiolipin.
The term antiphospholipid Ab includes the lupus anticoagulant and
anticardiolipin antibody.
C/P 1- Blood  plat  - Autoimm. H. An
2- Cardiac  myocardial infraction.
3- Neuro  cerebral infraction
4- Renal  renal vein thrombosis.
5- Endocr  adrenal thrombosis
6- GIT  bowel ischamia and Budd chiari $.
7- Obestatric  abortion.
Investing - Prolonged P.T.T
- Ab detection
 64

ttt * Cause * Anticoag

Blood transfusion reactions

Classification of transfusion reactions

(1) Acute (during or within 72 hrs)

 Immunological
 Haemolytic transfusion reaction.
 Non haemolytic
o Febrile reaction
o Allergic reaction.
o Non cardiac pulm edema
 Non Immunological
 Circulatory overload.
 Bacterial contaminations.
 Dilutional coag defects.
 Effects of stored blood e.g. hypothermia  O2
affinity.
(2) Delayed (after 72 hrs)
 Immunological
 Hemolytic reaction.
 Post transfusion purpura.
 Non Immunological
 Iron overload
 Viral hepatitis A, B, C
 HIV
 $ - malaria

Acute haemolysis
Due to incompatible red cells, usually in the ABO system, this occurs
within minutes.

C/P within minutes  Rigors and fever

 Lumbar pain
 Chest tightness
 Hypotension
 65

ttt
*Stop the transfusion.
* Re- group and cross match again
* Check blood count, bilirubin
* Monitor pulse, Bl. Pr
* Support pt’s circulatory state

Febrile non hemolytic transfusion

reactions
Due to antiwhite cell or anti HLA Abs reaction with white cells in
the unit.

C/P Fever – rigors – urticaria

ttt = Stop.
= Exclude haemolytic reaction
= Antipyretic
= Use of leucodepleted blood
= Steroids in further blood needed

Delayed hemolytic transfusion

reaction
This occurs 5-7 days after transfusion. The transfusion has
stimulated the production of an AB which was not detected at the initial
cross match.
C/P Anaemia + J

ttt = Ab detection and spherocytes.

= Compatible blood use.
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Blood components and products

ㄱ Whole Blood
The average of blood in 450 ml + 63 anticoagulant.
Stored at 4°C has shelf life of 5 weeks. Used for acute
blood loss.
ㄱ Packed red cells
200-250ml plasma are removed from whole blood.
Used in treatment of blood loss without causing
hypervolemia e.g. in heat diseases.
ㄱ Whashed red cell concentrates
Used in patients with history of severe recurrent
urticarial or anaphylactic reactions.
ㄱ Platelet concentrates
They may be stored for up to 5 days at 22°C. used for
cases of thrombocytopenia with bleeding.
ㄱ Granulocyte concentrate
For patients with severe neutropenia.
ㄱ Fresh frozen plasma
Is prepared by freezing the plasma from 1 unit of
blood at zero degree with 6 hrs of donation. The
volume about 200ml. Used for replacement of factors
of coagulation.
ㄱ Cryoprecipitate
Volume about 20ml, It contains factor VIII
ㄱ Human albumin
ㄱ Immunoglobulin
Prepared from plasma used to prevent infections. Also
other preparations e.g. anti-hepatitis B.

Myelodysplastic syndromes
The muyelodysplastic syndromes are characterized by the slow
development of an anemia refractory to standard therapy. This syndrome
has been called refractory anemia or preleukemia in the past.
C/P Anemia refractory to treatment
Investing * HB  * MCV  (macrocytic An)
ttt * ttt An * Blood transfusion
* Vit B6 in some cases
 67

Plasma cell disorders

The plasma disorders include a group of B cell neoplasms that arise from
a clone of immunoglobulin secreting cells and produce monoclonal
immunoglobulin.

If the Ig is of the IgM If the monoclonal Ig is of

Class the disease is The IgG, IgA, IgD or rarely
Waldenstrom’s IgE class the disease is
Macroglobulinemia Multiple myeloma

Multiple Myeloma
This is a malignant disease of plasma cells that is characterized by the presence of
monoclonal immunoglobulin in the serum, urine and bone destruction and the
malignant cells are plasma cells.
C/P *
Bone involvement:- malignant plasma cells may secrete
cytockines that activate osteoclasts leading to osteoprosis
and hypercalcemia.
* Anemia :- due to marrow invasion by plasma cell.
* Renal diseases :- Ca and uric acid nepropathy.
* Infection :- e.g. pneumonia.
* Bleeding (platelet function and count ).
* Hyperviscosity.
Investi + ESR (the paraprotein cause rouleaux)
ng
+ Serum electrophoresis  monoclonal Ig E.g. IgG
+ B.M ex (diagnostic) normally BM. Contains about 5% of
plasma cell in multiple m. plasma cells may reach greater
than 10-20%
+ Alkaline phosphatase enzyme is normal (there is no
stimulation of osteoblasts)
ttt * Bone lesions may require radiotherapy to prevent
pathological frs.
* Pneumocorcal vaccine.
* I.V. gamma globulin
* Cytotoxic drugs and steroids.
 68

* B.M transplantation.
Waldenstrom’s
macroglobulinemia
This is a clonal disease of IgM secreting plasmacytoid lymphocytes
usually it affects older people.
IgM is a large molecule and remains in the intravascular compartment, so
if  it will lead to hyperviscocity $.
C/P - Blue cyanotic fingers, toes, nose and earlobes on
exposure to cold.
- Nose bleeds, retinal Hge.
- Congestive HR. failure
- Foot, leg ulcers and vascular occlusion with gangrene.
- Raynaud’s phenomenon.
- Peripheral neuropathy.
- Electrophoresis reveals IgM 
Ttt * Plasmapharesis
* Cytotoxic drugs

B.M. transplantation
Indications: 1- Thalassaemia major
2- A plastic anaemia
3- Acute leukemia.
Method Multiple marrow aspirations are performed on the
iliac crests. The aspirated B.M. mixed with heparin  I.V.
infusion to the recipient.
Complications
 Inf. (herpes, CMV, fungal, pneumocystis. Carnii).
 Recurrence – effects of cytotoxic drugs.
 Graft versus host reaction.

Graft versus host reaction

It is due to the cytotxic activity of donor T lymphocytes which become
sensitized to their new host.
Acute GVHR It appears 14-21 days after the graft, it can
affect the skin, liver, and gut. IT can be treated by
cyclosporine, methotrexate and steroids.
 69

Chronic GVHR This may follow acute, It is similar to C.T

disease, with rach, it can be treated by steroids and
cyclosporin