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H,PC: recent update Dr. RAFIQ AHMAD
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Transcript of H,pc
H,PC: recent update
Dr. RAFIQ AHMAD
Donath-Landsteiner Hemolytic Anemia
Background 1st described in 1854 In the later part of 19th century- Congenital or
tertiary Syphilis Antibody discovered in 1902 Test was published in 1904, revised in 1990
Vox Sang 1990;58(4):281-6 Specificity to red cell P antigen in 1963
Pathophysiology Biphasic IgG antibody binding red cell P antigen
in cold at peripheral circulation and dissociates at 37 C at central circulation, leading to complement activation to C 9 and results in intravascular hemolysis
Hemolysis leads to anemia, hemoglobinuria, that may sometimes lead to renal failure
Immune Hemolytic AnemiasClassification
Autoimmune Hemolytic Anemia (AIHA) percentage Warm AIHA 48-70% Cold agglutinin disease 16-32% DLHA (H,PC) 32% in children Mixed-type AIHA 7-8% DAT-negative AIHAAlloimmune Hemolytic Anemia Hemolytic transfusion reaction Hemolytic disease of the fetus and newbornDrug-Induced Immune Hemolytic Anemia Drug-dependent Drug-independent
Causes Idiopathic Secondary children under the age of 5: post-viral infection (after 2-3 weeks) older children and adults : infectious, rare associated with neoplasm Br J Haematol 1999;105(1):278-9
Frequency 4 in 1 million (Sokel et.al ) Acta Haematol 1984;72(4): 245-7
Mortality/Morbidity Spontaneous recovery within days to weeks Prognosis is excellent In older patients severe anemia can lead to permanent
renal damage
Race No racial predisposition recognized
Sex Male to female ratio is 2-5:1 Transfusion 2007; 47(1) 50-4
Age Mainly a disease of children under the age of 5
Clinical PresentationSymptoms Generalized (within few minutes to few hours)
» back pain
» leg cramps
» chills
» headache
» passing of dark red urine Oliguria or even anuria Cold urticaria and jaundice
Signs Pallor Icterus Urticarial eruptions An exanthema related to primary viral disease Hemoglobinuria (passing of dark red or brown urine) Patients with lymphoma or neoplasm
- lymphadenopathy and splenomegaly
Differential diagnosis Cold agglutinin disease (CAD) Paroxysmal nocturnal hemoglobinuria (PNH) Malaria: P. falciparum Other AIHA Myoglobinuria Hemolytic transfusion reaction (HTR) Hemolytic uremic syndrome (HUS)
Laboratory studies
CBC » ↓Hb.
» ↑Retic count ( initially it may be low)
» ↓Leukocyte count, followed by
neutrophilic leukocytosis PBF » Spherocytosis
» Monocytes and granulocytes show
phagocysed RBC’s in acute event
Laboratory studies
Blood smear in CAD Blood smear in DLHA
Laboratory studies
Urine of a patient with PNH, showing the episodic nature of the dark
Urinanalysis
» Hemoglobinuria (dark or brown red color)
» Hemosidrin (if ongoing ch. Hemolysis)
Laboratory studies Biochemical
» ↑ LDH
» ↑ Indirect bilirubin
» ↓ heptoglobin
» presence of free Hb.
» ↓ Complement levels Test for infectious diseases
» viral
» syphilis
» Gram smear & culture
Laboratory studies Blood Bank
» Antiglobulin test
- DAT
- IAT
» D-L test
Modified D-L TestTubes I II III Incubation
A
2 drops of Pt. serum
+
1 drop of 50% of Cell sus
Reaction: + Hemolysis
2 drops of Pt. serum
+
1 drop of 50% of Cell sus
+
2 drops of fresh serum
Reaction: + Hemolysis
2 drops of fresh serum
+
1 drop of 50% of Cell sus
Reaction: 0
30 minutes inmelting ice
60 minutes at 37 C
B
2 drops of Pt. serum
+
1 drop of 50% of Cell sus
Reaction: 0
2 drops of Pt. serum
+
1 drop of 50% of Cell sus
+
2 drops of fresh serum
+
Reaction: 0
2 drops of fresh serum
+
1 drop of 50% of Cell sus
Reaction: 0
90 minutes in
melting ice
C
2 drops of Pt. serum
+
1 drop of 50% of Cell sus
Reaction: 0
2 drops of Pt. serum
+
1 drop of 50% of Cell sus
+
2 drops of fresh serum
+
Reaction: 0
2 drops of fresh serum
+
1 drop of 50% of Cell sus
Reaction: 0
90 minutes at 37 C
Laboratory studies Image studies FNAC Excisional biopsy of enlarged lymph node
Treatment Supportive care with hydration, and alkalization of
urine to prevent renal failure Treat underlying disease Antihistamine for urticaria Transfusion
Leukocyte reduced red cells
( could be red cell P Ag +)
frequency of p Ag is 1: 200,000 units
Consultation Folic acid supplement in chronic form of disease Encourage patient to eat fresh fruits and
vegetables. Avoiding of cold
Conclusion Close co-ordination among patient’s physician,
hematologist/ transfusion medicine specialist
The end!!
Thank you
