HEMOLYTIC ANEMIAS:-
 Normal life span of rbc is 120 days
 Premature destruction
 Increase in reticulocyte index
 Intravascular hemolysis
 Free Hb binds to haptoglobin
 Hemoglobinuria,hemoiderinuria
 Haptoglobin levels are reduced
 Extravascular hemolysis by liver and spleen
 Megaloblastosis due to folate deficiency
 Compensated and decompensated hemolysis
CLASSIFICATION :-
 Abnormalities of RBC interior
a. enzyme defects
b. hemoglobinobathies
 RBC membrane abnormalities
a. hereditary spherocytosis
b. paroxysmal nocturnal hemoglobinuria
c. spur cell anemia
 Extrinsic factors
a. hyperspleenism
b. antibody: immune hemolysis
c. microangiopathic hemolysis
d. infections, toxins etc
HEREDITARY SPHEROCYTOSIS :-
 inherited as autosomal dominant
 25% cases have no family history
 Common are deficiency of betaspectrin or
ankyrin
CLINICAL FEATURES :-
 Chronic haemolytic state
 Haemolytic crisis
 Megaloblastic crisis
 Anaplastic crisis : parvovirus B-19 suppresses
bonemarrow
 Symptomatic cholecystitis
INVESTIGATIONS :-
 CBC – spherocytes
 Direct coombs test – negative
 Osmotic fragility test – sensitivity to lysis in
hypotonic solution
 Flow cytometry – binding of eosin 5- maleimide
to rbc
TREATEMENT :-
 Folic acid supplement
 Spleenectomy
 Transfusion
HEREDITARY ELLIPTOCYTOSIS :-
 The G6PD gene located on x chromosome
 Pivotal in HMP shunt
 Produces NADPH to protect RBC cells
 Affects males
CLINICAL FEATURES :-
 Precipitating factors : antimalarials
 chronic compensated state
 favism ( toxin present in weeds )
 acute illness
INVESTIGATIONS :-
 CBC : elliptic rbc cells
 G6PD levels
TREATEMENT :-
 stopping precipitants
 transfusion support
WARM ANTIBODY IMMUNOHEMOLYTIC
ANEMIA :-
 mid age and females
 causes
a. idiopathic
b. lymphomas
c. SLE and other collagen vascular diseases
d.Drugs –methyl dopa type
Penicillin type
Quindine type
e. post viral infections
INVESTIGATIONS :-
 blood smear –hemolysis , reticulocytosis
 direct coombs test
TREATEMENT :-
 treating the cause
 prednisone
 transfusion
 spleenectomy
 immunosuppression
COLD AIHA :-
 active at lower body temperature
 IgM antibodies
 Elderly persons
 Underlying lymphoma
 Donath Landsteiner antibody
 Mycoplasma
TREATEMENT :-
 Treat the cause
 Steroids
PAROXYSMAL NOCTURNAL
HEMOGLOBINURIA :-
 An intra corpuscular defect acquired at the stem
cell level
 Inactivating somatic mutation in a single
hematopoietic stem cell of a gene on the x
chromosome (pig-A) for GPI
 Absence of CD55 and CD59
 Complement mediated hemolysis
CLINICAL FEATURES :-
 Haemolytic anemia
 Venous thrombosis
 Deficient hematopoiesis
INVESTIGATIONS :-
 Evidence of intravascular hemolysis ,
hemogolinemia, elevated LDH, hemosidenuria
 Leukopenia
 Thrombocytopenia
 Acidified serum lysis test
 Sucrose lysis test
 Flow cytometry
TREATEMENT :-
 Transfusion
 Glucocorticoids
 Acute thrombosis –anticoagulants
 Marrow transplantation
 Anti-thymocyte globulin – treating marrow
hypoplasia
APLASTIC ANEMIA :-
 Hypoproliferative anemia with marrow failure
a. aplastic anemia
b.myelodysplasia : normal stem cell to abnormal
premalignant cells leading to acute leukaemia
c. myelophthisis : bone marrow is normal and
there is infilteration of other cells.
Pancytopenia occurs
d. pure red cell aplasia
 pancytopenia – anemia
leukopenia
thrombocytopenia
 aplastic anemia – pancytopenia and bone
marrow hypocellularity
ETIOLOGY
 radiation – acute sequela
 chemicals – benzene
 drugs
a. dose dependent
 cancer chemotherapy
 alkylating aents, antimetabolites
 idiosyncratic
a. chloraphenicol , NSAIDS (long standing
osteoarthritis)
b. anticonvulsants, sulfanamides
 infections
a. hepatitis
b. infectious mononucleosis – EBV
c. parvovirus B19
 immunologic disorders
a. transfusion associated GVHD
b. SLE (collagen vascular disease)
 Paroxysmal nocturnal hematuria
 Congenital disorders
a. fanconi’s anemia
b. dyskeratosis congenital
c. shwachmann diamond syndrome
 pregnancy
CLINICAL FEATURES :-
 onset
a. abrupt
b. insidious
 bleeding most common early symptom
 anemia symptoms
 infection
 history of drug intake, chemical exposure or
preceding viral illness
 lymphadenopathy,splenomagaly absent
LAB STUDIES :-
 peripheral smear
 bone marrow
a. aspiration
b. biopsy – hematopoietic cells< 25% of marrow
space
TREATMENT :-
 bonemarrow transplantation – young patient
with fully histocompatible sibling donor
 restrict transfusion
 immunosuppression
a. anti lymphocyte globulin
b. anti thymocyte globulin
c. cyclosporine
 supportive therapy
a. antibiotics
b. transfusion support
MYELODYSPLASIA :-
 characterized by cytopenias
 dysmorphic (or abnormal appearing ) and
usually cellular bone marrow
 ineffective blood cell production
 occurs in elderly persons
 more in males
CLASSIFICATION :-
 refractory anemia (RA)
 refractory anemia with ringed
sideroblasts(RARS)
 refractory cytopenia with multilineage dysplasia
(RCMD)
 refractory anemia with excess blasts – 1 (RAEB –
1 and 2)
 myelodysplastic syndrome, unclassified
 MDS with isolated del (5q)
ETIOLOGY :-
 Radiation
 Benzene
 Alkylating agents such as busulfan, nitrosourea
or procarbazine
 Cytogenetic abnormalities – aneuploidy
CLINICAL FEATURES :-
 Asymptomatic – 50%
 Anemia
 Fever and weight loss- absent
 Splenomegaly
 Skin lesions - sweet’s syndrome
(febrile neutrophilic
dermatosis)
LAB STUDIES :-
BLOOD
 Anemia
 Bi or pancytopenia
 Isolated neutropenia or thrombocytopenia
 Macrocytosis is commom
 May be dimorphic
BONE MARROW
 Usually normal or hypercellular
 Dyserythropoietic changes and ringed
sideroblasts
 Hypogranulation and hyposegmentation in
granulocytic precursors
 Increase in myeloblasts
 Vitamin B12 or folate-normal
 Rule out viral infections, drug reaction and
chemical intoxicity
TREATMENT :-
 Unsatisfactory
 Stem cell transplantation – cure
 5 azacytidine inhibits DNA methylation
 G-CSF

 Supportive care

HALLPIKE MANOEUVRE

                                         
                        Video to show how Hallpike Manoeuvre is done.

Method

·       Patient sits on a couch

·       Examiner holds the patient’s head, turns it to 45^o to the right and then places the patient in a supine position so that his head hangs 30^o below the horizontal

·       Patient’s eyes are observed for nystagmus

·       The test is repeated with head turned towards left and then in straight head hanging position

  

·       Four parameters of nystagmus are observed – latency, duration, direction, fatiguability

·      

In BPPV            

      · Nystagmus appears after a latent period of 2- 20 seconds, lasts for 1 minute and is always in                                                    one direction

       · On repetition of the test, nystagmus may still be elicited but lasts for a shorter period

 ·   On subsequent repetitions, it disappears together, i.e. nystagmus is fatiguable                                                         

      ·   Patient also complains of vertigo when the head is in critical position

·  

In central lesions (tumors of IVth ventricle, cerebellum, temporal lobe, multiple sclerosis, vertebrobasilar insufficiency or raised intracranial lesion), nystagmus is produced immediately as soon as the head is in critical position without any latency and lasts as long as head is in that critical position 

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