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Bone Marrow Failure

This document discusses bone marrow failure, also known as aplastic anemia. It defines bone marrow failure as the specific failure of the bone marrow to produce normal numbers of mature blood cells, resulting in low blood cell counts. The document then classifies, discusses causes, pathology, clinical features, lab investigations, and treatment options for bone marrow failure. Treatment may include blood transfusions, antibiotics, immunosuppressive therapies like antithymocyte globulin, corticosteroids, and bone marrow transplantation.

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Isaac Mwangi
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88 views16 pages

Bone Marrow Failure

This document discusses bone marrow failure, also known as aplastic anemia. It defines bone marrow failure as the specific failure of the bone marrow to produce normal numbers of mature blood cells, resulting in low blood cell counts. The document then classifies, discusses causes, pathology, clinical features, lab investigations, and treatment options for bone marrow failure. Treatment may include blood transfusions, antibiotics, immunosuppressive therapies like antithymocyte globulin, corticosteroids, and bone marrow transplantation.

Uploaded by

Isaac Mwangi
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
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Download as PPT, PDF, TXT or read online on Scribd
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BONE MARROW

FAILURE
DR ALEX MOGERE
Consultant Physician
OBJECTIVES

 Definition
 Classification
 Aetiology
 Pathology
 Clinical features
 Lab Investigation
 Treatment
Definition

 Specific failure of bone marrow B cells to produce the


normal number of mature cells resulting in peripheral
blood cytopenias
 Bone marrow failure is also known as APLASTIC ANAEMIA
CLASSIFICATION

 Primary- Idiopathic
 Secondary
 – Exposure to toxic agents eg drugs, chemicals,
viruses
 _Associated with-Fanconi’s anemia, infective
hepatitis, pancreatic insufficiency, PNH, pure red cell
aplasia (both congenital & acquired)
AETIOLOGY

 1- Inevitable – Ionizing radiation & cytotoxic drugs


 2- Idiosyncratic – Idiopathic, drug induced, viruses
(hepatitis, EBV, HIV, HPV, Dengue fever, Parvovirus ),
some mycobacteria, commercial solvents
 3- Inherited-Fanconi’s, Dyskeratosis congenita,
diamond-blackfan anemia,others
 4- Industrial- Benzene
 5- Immune- Drug induced, viruses (EBV), SLE
 6- Infiltrations
 -infective- Leishmaniasis, miliary TB
 -storage disorders- Gaucher’s,
 -malignancies- lymphomas, plasma cell dyscrasias,
metastatic carcinomas, myeloproliferative disorders
both leukemic & non-leukemic
DRUGS STONGLY LINKED
TO APLASTIC ANAEMIA
 1 – Antibiotics – chloramphenicol, sulphonamides
including cotrimoxazole
 2 – NSAIDS – phenyl butazone, gold salts,
indomethacin, benoxaprofen, piroxicam
 3 –Antithyroids – thiouracils, carbimazole
 4 – Antimalarials – amodiaquine, pyrimethamine
 5 – Anticonvulsants – phenytoin
 6 – Antidepressants – chlorpromazine, prothiaden
PATHOLOGY

 Postulated mechanisms;

 1 – Qualitative Common Stem cell defect

 2 - Defective bone marrow microenvironment

 3 - Immune suppression
DDX OF ACQUIRED BMF

 PNH
 MDS
 Large granular lymphocytic leukemia
 Immune pancytopenias in CTD eg Refractory anemia in
SLE
 AML
 Hairy cell leukemia
 anemia
CLINICAL FEATURES

 Primary much less common than Secondary


 Primary occurs in all ages but rare in infants & children.
It maybe acute or chronic.
 Secondary occurs in all ages . It may be acute or chronic
Features due to Cytopenia

 1 – Anaemia – lassitude, fatiguability, dyspnoea

 2 – Leukopenia – fever, chills, mouth ulcers, recurrent


infections

 3 – Thrombocytopenia – echymoses, petechiae,


haemorrhage
Features due to cause

 Chemical toxicity – rash, pruritus, arthralgia


 Drugs – signs &symtoms of the original condition for
which the drug was used
 Marrow infiltrations - s & s of the infiltrative disease
 Features of the associated diseases
LABORATORY FEATURES
 1 – TBC – low Hb, low rbc ,normal indeces
 -low wbc – total with neutropenia
 - low platelets

 2 – PBF – normocytic normochromic with


 absent polychromasia

 3 – ESR – raised

 4 – BMA – hypoplastic/aplastic or a dry tap or


 marrow infiltrations

 5 - Trephine – reduced haemopoeitic tissue with


 increased fat spaces & stroma
 6 – Others – due to asociated conditions , infiltrative
conditions ,diseases

TREATMENT

 1 – SUPPORTIVE
 Anaemia – packed rbc if in failure
 Infections – relevant antibiotics
 Haemorrhage – platelet concentrates
 Splenectomy – is indicated if;
 -transfusion dependent or
 -not responding to therapy
2 – DEFINITIVE TREATMENT

 1 – BMT – young & HLA matched


 2 – Immunosuppression – ALG
 -highdose steroid
 -cytotoxics
 3 – Androgens – oxymetholone & steroids
 4 – Others – growth factors
 5 – Treatment of cause or original disease
Ct Tx

 ATG OR ALG should be given with corticosteroids to


prevent serum sickness
 High dose methylprednisolone (20mg/kg/day with rapid
taper), if ATG /ALG is expensive
 Cyclosporine therapy at a dose of 200-400mg/d has a
reported 85% hematologic remission rate
 Androgens are poorly tolerated by females and chidren(but
include testosterone proprionate,nandrolone
decanoate,oxymetholone etc)
 Danazol is a non masculinizing androgen that may be useful
 Hematopoietic growth factors eg G-csf,GM-csf in
neutropenic cases

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