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Auto immune hemolytic anemia (AIHA).pptx

Alfi, a 10-year old boy, presented with sudden onset of severe pallor, dark colored urine, and jaundice. Examination found severe pallor, jaundice, splenomegaly, and otherwise normal vital signs. Initial workup showed features of hemolysis, positive direct Coombs test, and elevated bilirubin and LDH. The provisional diagnosis was autoimmune hemolytic anemia, which was confirmed. Treatment with steroids led to improvement in symptoms and laboratory values over subsequent follow ups.

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WELCOME TO extended clinical meeting Presenters: DR. RENESHA ISLAM YEAR-4 RESIDENT (PHASE-B) PAEDIATRIC HAEMATOLOGY & ONCOLOGY DEPARTMENT
2 PARTICULARS OF THE PATIENT ▸ Name: Alfi Bin Hadi ▸ Age: 10 years ▸ Sex: Male ▸ Address: Basabo ▸ DOA : 13/03/21 ▸ DOE : 13/03/21 ▸ Informant: Mother & Himself
3 Presenting complaints ▸ Sudden onset of severe pallor for 10 days. ▸ Passage of dark colored urine for same duration. ▸ Yellow discoloration of whole body for 8 days.
4 History of present illness According to the statement of the patient himself and his mother, Alfi was reasonably well 10 days back. Then he developed severe pallor of sudden onset and passage of dark colored urine for same duration. He also developed yellow discoloration of eyes which gradually deepens and involved all over the skin including palms and soles for 8 days.
CONTINUED.. 5  On query, Mother mentioned that Alfi had a history of febrile episode and sore throat about 11 days prior to this illness which resolved spontaneously.  He had no history of abdominal pain, back pain, pruritus, skin rash, weight loss, arthralgia, bleeding manifestation, no history of taking any offending drugs, no family history of gall stone or such type of illness in his family.
CONT.. 6  For this reason, He got admitted in a private hospital and treated with oral prednisolone and 3 units of blood transfusion.  But his condition did not improved. So, With these above complaints, he was referred to BSMMU for proper evaluation and further management.
HISTORY OF PAST ILLNESS 7 • Alfi had a history of fever and sore throat about 11 days prior to this illness. • That time he only took oral antipyretics and it resolved spontaneously.
BIRTH HISTORY 8  Antenatal: Mother was on regular antenatal check up. She had no H/O Fever, Rash, HTN, DM, any features of Infections or taking any offending drugs.  Natal: Delivered at term by normal vaginal delivery with birth weight 3250 gm.  Postnatal : Uneventful. There was no H/O neonatal jaundice.
9 ▸ Developmental History: Age appropriate. He reads in class 4 with good academic performance. ▸ Immunization History: Completely Immunized as per EPI schedule.
10 ▸ Feeding History: He is on family diet. ▸ Travel History: Nothing significant.
FAMILY HISTORY 11 1st issue of his non consanguineous parents. He had no family history of such type of illness. His parents and only sib are in good health.
SOCIO-ECONOMIC HISTORY 12 ▸ He belongs to a upper middle class family. ▸ Father is a businessman and average monthly income is 60,000 taka. Mother is a home maker. ▸ They live in brick build building, drink purified water and use sanitary latrine.
TREATMENT HISTORY 13 ▸ He was treated with oral prednisolone 2mg/kg/day for 7 days during his admission period on that private hospital along with 3 units of PRBC transfusion.
GENERAL PHYSICAL EXAMINATION 14
GENERAL EXAMINATION 15 ▸ Appearance : Ill looking ▸ Pallor: Severe ▸ Jaundice: Moderate ▸ Cyanosis ▸ Edema ▸ Dehydration ▸ Koilonychia Absent ▸ Clubbing ▸ Leukonychia
CONT…. 16 ▸ Lymph nodes: Not palpable. ▸ Bony tenderness : Absent. ▸ Skin survey : Normal. ▸ BCG mark : Present. ▸ Ex of eye, ear, nose & throat: Normal. ▸ Vital signs ▸ Pulse : 112 beat/min. ▸ BP : 100/60 mm Hg (Both SBP& DBP lies between 50th and 90th centile) ▸ Respiratory Rate : 32 breaths/min. ▸ Temperature : 98.40F.
ANTRHOPOMETRY 17
Anthropometry 18 • Weight : 40kg (lies between 75th -90th centile) • Heigth : 138cm (lies on 50th centile) • BSA : 1.28 m2
SYSTEMIC EXAMINATION 19
HEMATOPOITIC SYSTEM 20 ▸ Pallor: Severely pale. ▸ Jaundice: Moderately icteric. ▸ Bony tenderness: Absent. ▸ Lymph node examination: No lymphadenopathy. ▸ Skin survey: Normal. ▸ Oral cavity: Healthy.
21 ▸ Liver: Not palpable. ▸ Spleen: -6 cm from left costal margin along it’s long axis -Firm in consistency -Splenic notch present.
CARDIOVASCULAR SYSTEM 22 ▸ Inspection: -No visible pulsation. ▸ Palpation: -Position of trachea: Central -Position of apex beat: 5th intercostal space just medial to the mid clavicular line. -Heart rate: 112 b/min -Thrill : Absent -P2 : Not palpable -Left parasternal heave: Absent.
23 CARDIOVASCULAR SYSTEM ▸ Auscultation: -1st and 2nd heart sounds are audible in all four areas. -There is no murmur.
24 RESPIRATORY SYSTEM Inspection: Respiratory Rate: 32 b/min Shape of the chest: Normal Chest Movement: Symmetrical. Palpation: Trachea: Centrally Placed Chest Expansibility: Symmetrical Vocal Fremitus: Normal.
25  Percussion: ○ Percussion Note: Resonant all over the chest.  Auscultation: ○ Breath Sound: Vesicular ○ No Added sound. RESPIRATORY SYSTEM
Alimentary system examination 26 Mouth & oral cavity: Healthy. Abdomen proper: Inspection: - Abdomen is distended. - Umbilicus is centrally placed. - No scar mark, visible pulsation or engorged veins. - Flanks are not full.
27 Alimentary system examination Palpation:  Abdomen is non-tender, soft.  Liver: Not palpable.  Spleen : -6cm from left costal margin along its long axis -Firm in consistency -Splenic notch present.  Fluid thrill: Absent.
28 Alimentary system examination ▸ Percussion: Percussion note tympanic. No shifting dullness. ▸ Auscultation: Bowel sound present.
GENITO-URINARY SYSTEM 29 ▸ Kidneys: Not ballotable ▸ Urinary bladder : Not palpable ▸ Genitalia : Male type ▸ Hernial orifices: Intact.
30 ▸ Higher cerebral function: Intact. ▸ Examination of cranial nerves: Intact ▸ Motor function: Bulk of muscle- normal Tone – Normal Power- Normal Jerks- Normal ▸ Sensory: Intact ▸ Cerebellar function: Intact. NERVOUS SYSTEM EXAMINATION
LOCOMOTOR SYSTEM EXAMINATION 31 ▸ LOOK: No joints are swollen, no periarticular wasting, No scar mark present. ▸ FEEL: Temperature- Normal Tenderness: Absent Fluctuation test- Not done Patellar tap – Not done ▸ MOVE: No restriction of movement.
SALIENT FEATURES 32 ▸ Alfi, 10 years old boy, 1st issue of his non consanguineous parents, immunized as per EPI schedule, got admitted with the complaints of sudden onset of severe pallor, high colored urine for 10 days & jaundice for 8 days. ▸ He had a history of febrile episode and sore throat about 11 days prior to this illness.
CONT…  Alfi had no history of fever, abdominal pain, back pain, pruritus, skin rash, no family history of gallstones or taking any offending drugs and none of his family members suffer from this sort of illness. 33
34 ▸ On examination- Alfi was ill looking, severely pale, moderately icteric, afebrile, Anthropometrically well thrived, vitals were within normal limit except tachycardia and tachypnea present. Abdomen was distended and splenomegaly present.
PROVISIONAL DIAGNOSIS 35
PROVISIONAL DIAGNOSIS 36 Hemolytic anemia (most probably auto immune hemolytic anemia)
DIFFERENTIAL DIAGNOSIS 37 ▸ Paroxysmal nocturnal hemoglobinuria. ▸ Glucose 6 phosphate dehydrogenase deficiency.
AUTO IMMUNE HEMOLYTIC ANEMIA 38 Points in Favor Sudden onset of severe pallor Yellow discoloration of whole body High colored urine Severe pallor Moderately icteric Splenomegaly
Paroxysmal nocturnal hemoglobinuria 39 Points in Favor Sudden onset of pallor Yellow discoloration of whole body High colored urine Severe pallor Moderately icteric Splenomegaly Points Against Urine discoloration not only in early morning or night No back pain No abdominal pain No easy bruising No hepatomegaly
G-6PD DEFICIENCY 40 Points in Favor Male child Sudden onset of pallor Yellow discoloration of whole body High colored urine Severe pallor Moderately icteric Splenomegaly Points Against No Family History No H/O Neonatal jaundice No hepatomegaly
investigations 41
INVESTIGATIONS DONE ON 06.03.21 42 ▸ CBC: Hb- 4.5 gm/dl Total count of WBC- 18,600/cmm Platelet count- 2,70,000/cmm ▸ PBF: Features of hemolysis. ▸ Reticulocyte count: 4.7% ▸ Hb electrophoresis: Normal ▸ S. Bilirubin (Total): 5 mg/dl ▸ S. ALT: 125 U/L ▸ S. LDH: 525 U/L ▸ PT: 12 sec ▸ APTT: 36 sec
CONT…. 43 ▸ Coomb’s test: Direct- Positive Indirect- Negative ▸ HBsAg: Negative ▸ Anti HCV: Negative ▸ IgG: 14.53 g/l (2.31-14.11 g/l) ▸ IgA: 0.95 g/l (.20-1.00 g/l) ▸ Anti-dsDNA: Negative ▸ ANA: Negative ▸ ICT Malaria: Negative ▸ Blood group: O (+ve)
INVESTIGATIONS DONE AFTER ADMISSION 44 ▸ CBC: Hb- 5 gm/dl TC of WBC- 17,170/cmm DC of WBC- N: 70% L: 20% M:5% E: 3% Platelet- 3,33,000/cmm MCV- 102 fL MCH- 38 pg MCHC- 30 g/dl ▸ Reticulocyte count: 8 %
CONT…. 45 ▸ PBF- -RBC: Anisocytosis, with many polychromatic cells, spherocytes, schistocytes, nRBC, tear drop cells. -WBC: Increased, mature with above count and distribution. -Platelet: Normal. ▸ Coomb’s test- Direct positive.
CONT… 46 ▸ S. bilirubin: Total- 5.5 mg/dl Direct- 0.8 mg/dl Indirect- 4.7 mg/dl ▸ SGPT: 50 U/l ▸ Prothombin time: Control-11.85sec Patient- 12.15 sec ▸ APTT: Control- 32.85 sec Patient- 30.15 sec ▸ INR: 1.08 ▸ Random blood sugar: 6.2 mmol/l
“ Auto immune hemolytic anemia 47 Final Diagnosis
TREATMENT 48 ▸ Counseling ▸ Inj. Methyl prednisolone: 4 mg/kg/dose, 6hrly for 5days ▸ Tab. Prednisolone: 2mg/kg/day ▸ Tab. Folic acid.
FOLLOW UP ON 14/03/2021 d-02 49 Subjective Objective Assessment Plan No new complaints Well alert Afebrile Moderately pale Icteric R/R- 22 b/min Pulse - 88 b/min, BP- 105/65 mmHg Lungs- Clear Heart- S1+S2+0 P/A/E- Soft, distended, Non-tender Spleen- 6 cm enlarged Bowel & bladder habit- Normal Improving CBC- • Hb- 7.8 gm/dl • TC of WBC- 12,200/cmm • DC of WBC- -N: 68% -L: 25% • Platelet- 2,29,000/cmm Continue same treatment
Follow up on 18/03/2021 (d-06) 50 Subjective Objective Assesment Plan No new complaints Well alert Ill looking Afebrile Mildly pale Anecteric R/R- 16 b/min H/R - 86 b/min, BP- 100/70 mmHg Lungs- Clear Heart- S1+S2+0 P/A/E- Soft, distended, Non-tender Spleen- 2 cm enlarged Bowel & bladder habit- Normal Improved CBC- • Hb- 11.9 gm/dl • TC of WBC- 10,012 /cmm • DC of WBC- -N: 69% -L: 25% • Platelet- 2,19,000/cmm Discharge with advice
51 4.5 5.5 6.2 6.8 6.5 5 7.8 9.2 10.3 11.9 12.5 13.5 13.7 13.5 13.7 0 2 4 6 8 10 12 14 16 Haemoglobin (gm/dl) date Oral prednisolone IV methyl pred Oral prednisolone Stop oral pred
52 4.7 8 4.2 2.86 2.4 1.77 1.07 1.05 1.02 0 1 2 3 4 5 6 7 8 9 Reticulocyte count % date Oral prednisolone Oral prednisolone IV methyl pred Stop oral pred
Auto immune hemolytic anemia 53
Causes of hemolytic anemia due to extra corpuscular defect 54 Extra corpuscular hemolytic anemia Immune Isoimmune Autoimmune Idiopathic (ex: warm antibody, cold antibody) Secondary Nonimmune Idiopathic Secondary
Auto immune hemolytic anemia 55 ▸ Autoimmune hemolytic anemia is a group of disorders characterized by a malfunction of the immune system that produces autoantibodies, which attack red blood cells as if they were substances foreign to the body. ▸ In AIHA shortened red cell survival is caused by the action of immunoglobulins, with or without the participation of complement on the red cell membrane.
incidence 56 ▸ Annual incidence: 1–3 cases/100,000. ▸ Approximately 0.2 cases/10,00,000 individuals under 20 years of age. ▸ Not race or gender specific. ▸ Slightly more likely to occur in females than males. WORLDWIDE PHO (BSMMU) (2019-2021 till now) 2 admitted 3 admitted
Causes of auto immune hemolytic anemia 57 1. Idiopathic Warm antibody Cold antibody Cold–warm hemolysis (Donath–Landsteiner antibody)
58 2) Secondary ○ Infection: Viral- EBV, CMV, Herpes simplex, Measles, Varicella, HIV. Bacterial- Streptococcal, E. coli, Mycoplasma. ○ Drugs and chemicals: Ceftriaxone, Penicillin, Quinine, Quinidine, Tetracycline, Rifampin, Sulfonamides. ○ Hematologic disorders: Leukemias, Lymphomas, Lymphoproliferative syndrome. ○ Immunopathic disorders: SLE, UC, Evans syndrome. ○ Tumors: Ovarian teratoma, Dermoids, lymphomas. Causes of auto immune hemolytic anemia
59 Mechanism of auto immune hemolytic anemia COLD ANTIBODY WARM ANTIBODY
60 CLINICAL FEATURES ▸ Clinical presentation & course may be either mild or more complicated & severe. ▸ The symptoms may be of acute or insidious onset. ▸ Most common: Sudden onset of pallor, jaundice, dark urine, splenomegaly, & hepatomegaly. ▸ Additional physical findings may be present when the hemolytic process is secondary to an underlying disorder .
Laboratory investigation 1st level tests: ▸ Complete blood count: Hemoglobin level: very low in fulminant disease or normal in indolent disease. Neutropenia and thrombocytopenia (occasionally). ▸ Reticulocyte count: Reticulocytosis common, although reticulocytopenia may occur. AIEOP guidelines for pediatric autoimmune hemolytic anemia
Peripheral blood film Nucleated RBC Spherocytes Polychromatophilic red cells Agglutination
Laboratory findings ▸ S. Bilirubin level: Hyperbilirubinemia (Indirect) ▸ S. lactate dehydrogenase: Increased. ▸ Haptoglobin level: Markedly decreased. ▸ Urinary urobilinogen: Increased. Hemoglobinuria especially at first presentation. ▸ Urinary hemosiderin. ▸ Osmotic fragility test: Increased and auto hemolysis proportional to spherocytes. ▸ Blood Grouping and Rh typing. ▸ Liver and Renal function test. AIEOP guidelines for pediatric autoimmune hemolytic anemia
▸ Extensive RBC typing in anticipation of possible transfusion ▸ Immune-hematological investigations: C3, C4, CH50 ▸ Auto-antibodies (ANA, anti DNA), anti-ph antibodies, RA test - Thyroid function and anti-thyroid antibodies - Lymphocyte sub populations (CD3, CD4, CD8, CD19, CD16) - Double-ve T cells: CD3+, CD4-, CD8- ▸ HBV & HCV markers and HIV serology ▸ Coagulation screen blood test ▸ Serum total protein and protein electrophoresis ▸ Immunoglobulin class quantification ▸ C-reactive protein ▸ EBV, CMV, Parvovirus B19, HSV serology ▸ Other assessments for infectious diseases (clinically appropriate) Second level tests AIEOP guidelines for pediatric autoimmune hemolytic anemia
Table : Characteristics of various forms of AIHA Clinical form Frequency % DAT Ig class Thermal Optimum (°C) Avidity and ability to fix comple- ment Anti- gen Specifi- city Site of heamolysis Warm antibody 60-70 IgG+ or IgG/ C3d+ IgG 34-37 -/+ Anti- Rh Extra- vascular Cold antibody 20-25 Neg or C3d+ IgM 4-27 +++ Anti-I Extra- vascular and intra- vascular Cold paroxysmal hemoglobi- nuria 6-12 Neg or C3d+ IgG Biphasic Fixing 4-27 Lysis 34-37 +++ Anti-p Intra vascular Mixed AEA <5 IgG+ or IgG/ C3d+ or C3d- IgG/ IgM IgG 34-37 IgM 4-27 ++ Anti- Rh Anti-I Extra- vascular and intra- vascular AIEOP guidelines for pediatric autoimmune hemolytic anemia
management Blood Transfusion: ▸ Transfusion should be avoided. ▸ Nonetheless, using the “least income- -patible” blood may be required in properly selected situations in order to avoid cardiopulmonary compromise. Conditions- -Washed packed red cells should be used from donors whose erythrocytes show the least agglutination in the patient’s serum. -Usually aliquots of 5 ml/kg are taken from a single unit and transfused at a rate of 2 ml/kg/h. -Concomitant use of high-dose corticosteroid therapy.
FIRST LINE THERAPY Oral prednisolone (1-2mg/kg/day for 3wks) Continue for 1 week Slow tapering (6 month) Prednisolone dependence Consider different diagnosis 2ND Line treatment NR Relapse CR-PR CR NR PR >0.1-0.2mg/kg/day Increase prednisolone back to previous dose Relapse <0.1-0.2mg/kg/day Continue at minimum effective dose Stop therapy CR Continue at full dose for 2 weeks PR AIEOP guidelines for pediatric autoimmune hemolytic anemia Eventual additional treatment in severe cases. eg. Methyl pred, IVIG, Plasma exchange
Second line THERAPY AIEOP guidelines for pediatric autoimmune hemolytic anemia
MONITORING This is potentially a life-threatening condition, So following must be monitored carefully: ▸ Hemoglobin level (every 4 hours) ▸ Reticulocyte count (daily) ▸ Splenic size (daily) ▸ Hemoglobinuria (daily) ▸ Haptoglobin level (weekly) ▸ Direct antiglobulin test (DAT) (weekly) Close attention should always be paid to supportive care issues such as folic acid supplementation, hydration status, urine output and cardiac status.
Clinical course 2 major groups of children with AIHA ▸ Acute course (50–70%) ▸ Chronic course (30–50%) 72
Prognosis 73 ▸ Unpredictable. ▸ Response rate to glucocorticoids is 68.6% in acute phase & even after relapse, 56.3% cases achieved complete remission with glucocorticoids only. This finding confirmed that steroid is the main stay of treatment. ▸ Relapse usually occurs 0.5 to 36 months after initial diagnosis with a median time of 4 months.
Mortality ▸ The adult mortality rate in one study was significantly higher (28.7%) than the corresponding pediatric rate (11%). ▸ When mortality occurs in children with AIHA it almost always is seen in those with a chronic presentation. ▸ The cause of death is rarely from the anemia; more commonly it is a complication of therapy or the underlying medical condition. 74
SARS-COV-2 & AIHA ▸ Two previously healthy children, each diagnosed with an autoimmune cytopenia associated temporarily with SARS-CoV-2 viral infection, one with acute ITP and one with AIHA. ▸ Here, neither exhibited symptoms of acute infection with SARS-CoV-2 prior to or at the time of presentation. 75 ▸ They suggested that SARS CoV-2 can trigger AIHA in predisposed children. ▸ In the current epidemiologic situation, upon the finding of severe hemolytic anemia without any apparent cause in a previously healthy child, SARS CoV-2 infection should be ruled out.
THANK YOU ALL….. 76

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Auto immune hemolytic anemia (AIHA).pptx

  • 1.
    WELCOME TO extended clinicalmeeting Presenters: DR. RENESHA ISLAM YEAR-4 RESIDENT (PHASE-B) PAEDIATRIC HAEMATOLOGY & ONCOLOGY DEPARTMENT
  • 2.
    2 PARTICULARS OF THEPATIENT ▸ Name: Alfi Bin Hadi ▸ Age: 10 years ▸ Sex: Male ▸ Address: Basabo ▸ DOA : 13/03/21 ▸ DOE : 13/03/21 ▸ Informant: Mother & Himself
  • 3.
    3 Presenting complaints ▸ Suddenonset of severe pallor for 10 days. ▸ Passage of dark colored urine for same duration. ▸ Yellow discoloration of whole body for 8 days.
  • 4.
    4 History of presentillness According to the statement of the patient himself and his mother, Alfi was reasonably well 10 days back. Then he developed severe pallor of sudden onset and passage of dark colored urine for same duration. He also developed yellow discoloration of eyes which gradually deepens and involved all over the skin including palms and soles for 8 days.
  • 5.
    CONTINUED.. 5  On query,Mother mentioned that Alfi had a history of febrile episode and sore throat about 11 days prior to this illness which resolved spontaneously.  He had no history of abdominal pain, back pain, pruritus, skin rash, weight loss, arthralgia, bleeding manifestation, no history of taking any offending drugs, no family history of gall stone or such type of illness in his family.
  • 6.
    CONT.. 6  For thisreason, He got admitted in a private hospital and treated with oral prednisolone and 3 units of blood transfusion.  But his condition did not improved. So, With these above complaints, he was referred to BSMMU for proper evaluation and further management.
  • 7.
    HISTORY OF PASTILLNESS 7 • Alfi had a history of fever and sore throat about 11 days prior to this illness. • That time he only took oral antipyretics and it resolved spontaneously.
  • 8.
    BIRTH HISTORY 8  Antenatal:Mother was on regular antenatal check up. She had no H/O Fever, Rash, HTN, DM, any features of Infections or taking any offending drugs.  Natal: Delivered at term by normal vaginal delivery with birth weight 3250 gm.  Postnatal : Uneventful. There was no H/O neonatal jaundice.
  • 9.
    9 ▸ Developmental History: Ageappropriate. He reads in class 4 with good academic performance. ▸ Immunization History: Completely Immunized as per EPI schedule.
  • 10.
    10 ▸ Feeding History: Heis on family diet. ▸ Travel History: Nothing significant.
  • 11.
    FAMILY HISTORY 11 1st issueof his non consanguineous parents. He had no family history of such type of illness. His parents and only sib are in good health.
  • 12.
    SOCIO-ECONOMIC HISTORY 12 ▸ Hebelongs to a upper middle class family. ▸ Father is a businessman and average monthly income is 60,000 taka. Mother is a home maker. ▸ They live in brick build building, drink purified water and use sanitary latrine.
  • 13.
    TREATMENT HISTORY 13 ▸ Hewas treated with oral prednisolone 2mg/kg/day for 7 days during his admission period on that private hospital along with 3 units of PRBC transfusion.
  • 14.
  • 15.
    GENERAL EXAMINATION 15 ▸ Appearance: Ill looking ▸ Pallor: Severe ▸ Jaundice: Moderate ▸ Cyanosis ▸ Edema ▸ Dehydration ▸ Koilonychia Absent ▸ Clubbing ▸ Leukonychia
  • 16.
    CONT…. 16 ▸ Lymph nodes:Not palpable. ▸ Bony tenderness : Absent. ▸ Skin survey : Normal. ▸ BCG mark : Present. ▸ Ex of eye, ear, nose & throat: Normal. ▸ Vital signs ▸ Pulse : 112 beat/min. ▸ BP : 100/60 mm Hg (Both SBP& DBP lies between 50th and 90th centile) ▸ Respiratory Rate : 32 breaths/min. ▸ Temperature : 98.40F.
  • 17.
  • 18.
    Anthropometry 18 • Weight :40kg (lies between 75th -90th centile) • Heigth : 138cm (lies on 50th centile) • BSA : 1.28 m2
  • 19.
  • 20.
    HEMATOPOITIC SYSTEM 20 ▸ Pallor:Severely pale. ▸ Jaundice: Moderately icteric. ▸ Bony tenderness: Absent. ▸ Lymph node examination: No lymphadenopathy. ▸ Skin survey: Normal. ▸ Oral cavity: Healthy.
  • 21.
    21 ▸ Liver: Notpalpable. ▸ Spleen: -6 cm from left costal margin along it’s long axis -Firm in consistency -Splenic notch present.
  • 22.
    CARDIOVASCULAR SYSTEM 22 ▸ Inspection: -Novisible pulsation. ▸ Palpation: -Position of trachea: Central -Position of apex beat: 5th intercostal space just medial to the mid clavicular line. -Heart rate: 112 b/min -Thrill : Absent -P2 : Not palpable -Left parasternal heave: Absent.
  • 23.
    23 CARDIOVASCULAR SYSTEM ▸ Auscultation: -1stand 2nd heart sounds are audible in all four areas. -There is no murmur.
  • 24.
    24 RESPIRATORY SYSTEM Inspection: Respiratory Rate:32 b/min Shape of the chest: Normal Chest Movement: Symmetrical. Palpation: Trachea: Centrally Placed Chest Expansibility: Symmetrical Vocal Fremitus: Normal.
  • 25.
    25  Percussion: ○ PercussionNote: Resonant all over the chest.  Auscultation: ○ Breath Sound: Vesicular ○ No Added sound. RESPIRATORY SYSTEM
  • 26.
    Alimentary system examination 26 Mouth& oral cavity: Healthy. Abdomen proper: Inspection: - Abdomen is distended. - Umbilicus is centrally placed. - No scar mark, visible pulsation or engorged veins. - Flanks are not full.
  • 27.
    27 Alimentary system examination Palpation: Abdomen is non-tender, soft.  Liver: Not palpable.  Spleen : -6cm from left costal margin along its long axis -Firm in consistency -Splenic notch present.  Fluid thrill: Absent.
  • 28.
    28 Alimentary system examination ▸Percussion: Percussion note tympanic. No shifting dullness. ▸ Auscultation: Bowel sound present.
  • 29.
    GENITO-URINARY SYSTEM 29 ▸ Kidneys:Not ballotable ▸ Urinary bladder : Not palpable ▸ Genitalia : Male type ▸ Hernial orifices: Intact.
  • 30.
    30 ▸ Higher cerebralfunction: Intact. ▸ Examination of cranial nerves: Intact ▸ Motor function: Bulk of muscle- normal Tone – Normal Power- Normal Jerks- Normal ▸ Sensory: Intact ▸ Cerebellar function: Intact. NERVOUS SYSTEM EXAMINATION
  • 31.
    LOCOMOTOR SYSTEM EXAMINATION 31 ▸LOOK: No joints are swollen, no periarticular wasting, No scar mark present. ▸ FEEL: Temperature- Normal Tenderness: Absent Fluctuation test- Not done Patellar tap – Not done ▸ MOVE: No restriction of movement.
  • 32.
    SALIENT FEATURES 32 ▸ Alfi,10 years old boy, 1st issue of his non consanguineous parents, immunized as per EPI schedule, got admitted with the complaints of sudden onset of severe pallor, high colored urine for 10 days & jaundice for 8 days. ▸ He had a history of febrile episode and sore throat about 11 days prior to this illness.
  • 33.
    CONT…  Alfi hadno history of fever, abdominal pain, back pain, pruritus, skin rash, no family history of gallstones or taking any offending drugs and none of his family members suffer from this sort of illness. 33
  • 34.
    34 ▸ On examination-Alfi was ill looking, severely pale, moderately icteric, afebrile, Anthropometrically well thrived, vitals were within normal limit except tachycardia and tachypnea present. Abdomen was distended and splenomegaly present.
  • 35.
  • 36.
    PROVISIONAL DIAGNOSIS 36 Hemolytic anemia (mostprobably auto immune hemolytic anemia)
  • 37.
    DIFFERENTIAL DIAGNOSIS 37 ▸ Paroxysmalnocturnal hemoglobinuria. ▸ Glucose 6 phosphate dehydrogenase deficiency.
  • 38.
    AUTO IMMUNE HEMOLYTICANEMIA 38 Points in Favor Sudden onset of severe pallor Yellow discoloration of whole body High colored urine Severe pallor Moderately icteric Splenomegaly
  • 39.
    Paroxysmal nocturnal hemoglobinuria 39 Points in Favor Suddenonset of pallor Yellow discoloration of whole body High colored urine Severe pallor Moderately icteric Splenomegaly Points Against Urine discoloration not only in early morning or night No back pain No abdominal pain No easy bruising No hepatomegaly
  • 40.
    G-6PD DEFICIENCY 40 Points in Favor Male child Suddenonset of pallor Yellow discoloration of whole body High colored urine Severe pallor Moderately icteric Splenomegaly Points Against No Family History No H/O Neonatal jaundice No hepatomegaly
  • 41.
  • 42.
    INVESTIGATIONS DONE ON06.03.21 42 ▸ CBC: Hb- 4.5 gm/dl Total count of WBC- 18,600/cmm Platelet count- 2,70,000/cmm ▸ PBF: Features of hemolysis. ▸ Reticulocyte count: 4.7% ▸ Hb electrophoresis: Normal ▸ S. Bilirubin (Total): 5 mg/dl ▸ S. ALT: 125 U/L ▸ S. LDH: 525 U/L ▸ PT: 12 sec ▸ APTT: 36 sec
  • 43.
    CONT…. 43 ▸ Coomb’s test:Direct- Positive Indirect- Negative ▸ HBsAg: Negative ▸ Anti HCV: Negative ▸ IgG: 14.53 g/l (2.31-14.11 g/l) ▸ IgA: 0.95 g/l (.20-1.00 g/l) ▸ Anti-dsDNA: Negative ▸ ANA: Negative ▸ ICT Malaria: Negative ▸ Blood group: O (+ve)
  • 44.
    INVESTIGATIONS DONE AFTERADMISSION 44 ▸ CBC: Hb- 5 gm/dl TC of WBC- 17,170/cmm DC of WBC- N: 70% L: 20% M:5% E: 3% Platelet- 3,33,000/cmm MCV- 102 fL MCH- 38 pg MCHC- 30 g/dl ▸ Reticulocyte count: 8 %
  • 45.
    CONT…. 45 ▸ PBF- -RBC: Anisocytosis,with many polychromatic cells, spherocytes, schistocytes, nRBC, tear drop cells. -WBC: Increased, mature with above count and distribution. -Platelet: Normal. ▸ Coomb’s test- Direct positive.
  • 46.
    CONT… 46 ▸ S. bilirubin:Total- 5.5 mg/dl Direct- 0.8 mg/dl Indirect- 4.7 mg/dl ▸ SGPT: 50 U/l ▸ Prothombin time: Control-11.85sec Patient- 12.15 sec ▸ APTT: Control- 32.85 sec Patient- 30.15 sec ▸ INR: 1.08 ▸ Random blood sugar: 6.2 mmol/l
  • 47.
  • 48.
    TREATMENT 48 ▸ Counseling ▸ Inj.Methyl prednisolone: 4 mg/kg/dose, 6hrly for 5days ▸ Tab. Prednisolone: 2mg/kg/day ▸ Tab. Folic acid.
  • 49.
    FOLLOW UP ON14/03/2021 d-02 49 Subjective Objective Assessment Plan No new complaints Well alert Afebrile Moderately pale Icteric R/R- 22 b/min Pulse - 88 b/min, BP- 105/65 mmHg Lungs- Clear Heart- S1+S2+0 P/A/E- Soft, distended, Non-tender Spleen- 6 cm enlarged Bowel & bladder habit- Normal Improving CBC- • Hb- 7.8 gm/dl • TC of WBC- 12,200/cmm • DC of WBC- -N: 68% -L: 25% • Platelet- 2,29,000/cmm Continue same treatment
  • 50.
    Follow up on18/03/2021 (d-06) 50 Subjective Objective Assesment Plan No new complaints Well alert Ill looking Afebrile Mildly pale Anecteric R/R- 16 b/min H/R - 86 b/min, BP- 100/70 mmHg Lungs- Clear Heart- S1+S2+0 P/A/E- Soft, distended, Non-tender Spleen- 2 cm enlarged Bowel & bladder habit- Normal Improved CBC- • Hb- 11.9 gm/dl • TC of WBC- 10,012 /cmm • DC of WBC- -N: 69% -L: 25% • Platelet- 2,19,000/cmm Discharge with advice
  • 51.
    51 4.5 5.5 6.2 6.8 6.5 5 7.8 9.2 10.3 11.9 12.5 13.5 13.7 13.513.7 0 2 4 6 8 10 12 14 16 Haemoglobin (gm/dl) date Oral prednisolone IV methyl pred Oral prednisolone Stop oral pred
  • 52.
    52 4.7 8 4.2 2.86 2.4 1.77 1.07 1.05 1.02 0 1 2 3 4 5 6 7 8 9 Reticulocytecount % date Oral prednisolone Oral prednisolone IV methyl pred Stop oral pred
  • 53.
  • 54.
    Causes of hemolyticanemia due to extra corpuscular defect 54 Extra corpuscular hemolytic anemia Immune Isoimmune Autoimmune Idiopathic (ex: warm antibody, cold antibody) Secondary Nonimmune Idiopathic Secondary
  • 55.
    Auto immune hemolyticanemia 55 ▸ Autoimmune hemolytic anemia is a group of disorders characterized by a malfunction of the immune system that produces autoantibodies, which attack red blood cells as if they were substances foreign to the body. ▸ In AIHA shortened red cell survival is caused by the action of immunoglobulins, with or without the participation of complement on the red cell membrane.
  • 56.
    incidence 56 ▸ Annual incidence:1–3 cases/100,000. ▸ Approximately 0.2 cases/10,00,000 individuals under 20 years of age. ▸ Not race or gender specific. ▸ Slightly more likely to occur in females than males. WORLDWIDE PHO (BSMMU) (2019-2021 till now) 2 admitted 3 admitted
  • 57.
    Causes of autoimmune hemolytic anemia 57 1. Idiopathic Warm antibody Cold antibody Cold–warm hemolysis (Donath–Landsteiner antibody)
  • 58.
    58 2) Secondary ○ Infection: Viral-EBV, CMV, Herpes simplex, Measles, Varicella, HIV. Bacterial- Streptococcal, E. coli, Mycoplasma. ○ Drugs and chemicals: Ceftriaxone, Penicillin, Quinine, Quinidine, Tetracycline, Rifampin, Sulfonamides. ○ Hematologic disorders: Leukemias, Lymphomas, Lymphoproliferative syndrome. ○ Immunopathic disorders: SLE, UC, Evans syndrome. ○ Tumors: Ovarian teratoma, Dermoids, lymphomas. Causes of auto immune hemolytic anemia
  • 59.
    59 Mechanism of autoimmune hemolytic anemia COLD ANTIBODY WARM ANTIBODY
  • 60.
    60 CLINICAL FEATURES ▸ Clinicalpresentation & course may be either mild or more complicated & severe. ▸ The symptoms may be of acute or insidious onset. ▸ Most common: Sudden onset of pallor, jaundice, dark urine, splenomegaly, & hepatomegaly. ▸ Additional physical findings may be present when the hemolytic process is secondary to an underlying disorder .
  • 61.
    Laboratory investigation 1st leveltests: ▸ Complete blood count: Hemoglobin level: very low in fulminant disease or normal in indolent disease. Neutropenia and thrombocytopenia (occasionally). ▸ Reticulocyte count: Reticulocytosis common, although reticulocytopenia may occur. AIEOP guidelines for pediatric autoimmune hemolytic anemia
  • 62.
    Peripheral blood film NucleatedRBC Spherocytes Polychromatophilic red cells Agglutination
  • 65.
    Laboratory findings ▸ S.Bilirubin level: Hyperbilirubinemia (Indirect) ▸ S. lactate dehydrogenase: Increased. ▸ Haptoglobin level: Markedly decreased. ▸ Urinary urobilinogen: Increased. Hemoglobinuria especially at first presentation. ▸ Urinary hemosiderin. ▸ Osmotic fragility test: Increased and auto hemolysis proportional to spherocytes. ▸ Blood Grouping and Rh typing. ▸ Liver and Renal function test. AIEOP guidelines for pediatric autoimmune hemolytic anemia
  • 66.
    ▸ Extensive RBCtyping in anticipation of possible transfusion ▸ Immune-hematological investigations: C3, C4, CH50 ▸ Auto-antibodies (ANA, anti DNA), anti-ph antibodies, RA test - Thyroid function and anti-thyroid antibodies - Lymphocyte sub populations (CD3, CD4, CD8, CD19, CD16) - Double-ve T cells: CD3+, CD4-, CD8- ▸ HBV & HCV markers and HIV serology ▸ Coagulation screen blood test ▸ Serum total protein and protein electrophoresis ▸ Immunoglobulin class quantification ▸ C-reactive protein ▸ EBV, CMV, Parvovirus B19, HSV serology ▸ Other assessments for infectious diseases (clinically appropriate) Second level tests AIEOP guidelines for pediatric autoimmune hemolytic anemia
  • 67.
    Table : Characteristicsof various forms of AIHA Clinical form Frequency % DAT Ig class Thermal Optimum (°C) Avidity and ability to fix comple- ment Anti- gen Specifi- city Site of heamolysis Warm antibody 60-70 IgG+ or IgG/ C3d+ IgG 34-37 -/+ Anti- Rh Extra- vascular Cold antibody 20-25 Neg or C3d+ IgM 4-27 +++ Anti-I Extra- vascular and intra- vascular Cold paroxysmal hemoglobi- nuria 6-12 Neg or C3d+ IgG Biphasic Fixing 4-27 Lysis 34-37 +++ Anti-p Intra vascular Mixed AEA <5 IgG+ or IgG/ C3d+ or C3d- IgG/ IgM IgG 34-37 IgM 4-27 ++ Anti- Rh Anti-I Extra- vascular and intra- vascular AIEOP guidelines for pediatric autoimmune hemolytic anemia
  • 68.
    management Blood Transfusion: ▸ Transfusionshould be avoided. ▸ Nonetheless, using the “least income- -patible” blood may be required in properly selected situations in order to avoid cardiopulmonary compromise. Conditions- -Washed packed red cells should be used from donors whose erythrocytes show the least agglutination in the patient’s serum. -Usually aliquots of 5 ml/kg are taken from a single unit and transfused at a rate of 2 ml/kg/h. -Concomitant use of high-dose corticosteroid therapy.
  • 69.
    FIRST LINE THERAPY Oralprednisolone (1-2mg/kg/day for 3wks) Continue for 1 week Slow tapering (6 month) Prednisolone dependence Consider different diagnosis 2ND Line treatment NR Relapse CR-PR CR NR PR >0.1-0.2mg/kg/day Increase prednisolone back to previous dose Relapse <0.1-0.2mg/kg/day Continue at minimum effective dose Stop therapy CR Continue at full dose for 2 weeks PR AIEOP guidelines for pediatric autoimmune hemolytic anemia Eventual additional treatment in severe cases. eg. Methyl pred, IVIG, Plasma exchange
  • 70.
    Second line THERAPY AIEOPguidelines for pediatric autoimmune hemolytic anemia
  • 71.
    MONITORING This is potentiallya life-threatening condition, So following must be monitored carefully: ▸ Hemoglobin level (every 4 hours) ▸ Reticulocyte count (daily) ▸ Splenic size (daily) ▸ Hemoglobinuria (daily) ▸ Haptoglobin level (weekly) ▸ Direct antiglobulin test (DAT) (weekly) Close attention should always be paid to supportive care issues such as folic acid supplementation, hydration status, urine output and cardiac status.
  • 72.
    Clinical course 2 majorgroups of children with AIHA ▸ Acute course (50–70%) ▸ Chronic course (30–50%) 72
  • 73.
    Prognosis 73 ▸ Unpredictable. ▸ Responserate to glucocorticoids is 68.6% in acute phase & even after relapse, 56.3% cases achieved complete remission with glucocorticoids only. This finding confirmed that steroid is the main stay of treatment. ▸ Relapse usually occurs 0.5 to 36 months after initial diagnosis with a median time of 4 months.
  • 74.
    Mortality ▸ The adultmortality rate in one study was significantly higher (28.7%) than the corresponding pediatric rate (11%). ▸ When mortality occurs in children with AIHA it almost always is seen in those with a chronic presentation. ▸ The cause of death is rarely from the anemia; more commonly it is a complication of therapy or the underlying medical condition. 74
  • 75.
    SARS-COV-2 & AIHA ▸Two previously healthy children, each diagnosed with an autoimmune cytopenia associated temporarily with SARS-CoV-2 viral infection, one with acute ITP and one with AIHA. ▸ Here, neither exhibited symptoms of acute infection with SARS-CoV-2 prior to or at the time of presentation. 75 ▸ They suggested that SARS CoV-2 can trigger AIHA in predisposed children. ▸ In the current epidemiologic situation, upon the finding of severe hemolytic anemia without any apparent cause in a previously healthy child, SARS CoV-2 infection should be ruled out.
  • 76.