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Simultaneous Pure Red Cell Aplasia and Auto-Immune Hemolytic Anemia in a Previously Healthy Infant

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Simultaneous Pure Red Cell Aplasia and Auto-Immune Hemolytic Anemia in a Previously Healthy Infant Simultaneous Pure Red Cell Aplasia and Auto-immune Hemolytic Anemia in a Previously Healthy Infant Robert P. Sanders, MD July 16, 2002 Case Presentation Patient Z.H. • Previously Healthy 7 month old WM • Presented to local ER 6/30 with 1 wk of decreased activity and appetite, low grade temp, 2 day h/o pallor. • Noted to have severe anemia, transferred to LeBonheur • Review of Systems – ? Single episode of dark urine – 4 yo sister diagnosed with Fifth disease 1 wk prior to onset of symptoms, cousin later also diagnosed with Fifth disease – Otherwise negative ROS •PMH – Term, no complications – Normal Newborn Screen – Hospitalized 12/01 with RSV • Medications - None • Allergies - NKDA • FH - Both parents have Hepatitis C (pt negative) • SH - Lives with Mom, 4 yo sister • Development Normal Physical Exam • 37.2 167 33 84/19 9.3kg • Gen - Alert, pale, sl yellow skin tone, NAD •HEENT -No scleral icterus • CHEST - Clear • CV - RRR, II/VI SEM at LLSB • ABD - Soft, BS+, no HSM • SKIN - No Rash • NEURO - No Focal Deficits Labs •CBC – WBC 20,400 • 58% PMN 37% Lymph 4% Mono 1 % Eo – Hgb 3.4 • MCV 75 MCHC 38.0 MCH 28.4 – Platelets 409,000 • Retic 0.5% • Smear - Sl anisocytosis, Sl hypochromia, Mod microcytes, Sl toxic granulation • G6PD Assay 16.6 U/g Hb (nl 4.6-13.5) • DAT, Broad Spectrum Positive – IgG negative – C3b, C3d weakly positive • Chemistries – Total Bili 2.0 – Uric Acid 4.8 –LDH 949 • Urinalysis Negative, Urobilinogen 0.2 • Blood and Urine cultures negative What is your differential diagnosis? Differential Diagnosis • Transient Erythroblastopenia of Childhood • Diamond-Blackfan syndrome • Underlying red cell disorder with Parvovirus induced Transient Aplastic Crisis • Immunohemolytic anemia with reticulocytopenia Hospital Course • Admitted to ICU for observation, transferred to floor 7/1. • 7/2 - Hgb 6.8 post transfusion. • 7/3 - Hgb 4.3, Bili 9.2 – Repeat DAT weakly positive for IgG and C3b,d – Indirect Coombs negative Hospital Course • Required repeat transfusions on 7/3, 7/4, 7/5, 7/7, and 7/8 to maintain Hgb > 7.0 • Solu-Medrol 2mg/kg/day started 7/3 • IVIG 1g/kg infused 7/8 • By 7/10 Hb stable at 9.3, Retic 4.8%, patient discharged on 1mg/kg/day prednisolone • Nasal swab culture positive for enterovirus Pure Red Cell Aplasia (PRCA) What are the forms of pure red cell aplasia found in pediatrics? Congenital PRCA (Diamond-Blackfan Anemia) • Normocytic to Macrocytic Anemia • 90% present in 1st year of life • Normocellular marrow with selective deficiency of erythroid precursors • Normal to increased leukocytes and platelets • Elevated erythropoietin, red cell adenosine deaminase, fetal hemoglobin Diamond-Blackfan • Physical Abnormalities in 24-30% – Short Stature – Craniofacial – Upper limb – Cardiac • 80% of cases sporadic • Pathophysiology – reduced or absent BFU-E or CFU-E – hyporesponsiveness to EPO • Leukemia develops in 2% Diamond - Blackfan • Treatment – Steroids • Rapid response without relapse 5% • Intermittent response 5% • Response with steroid dependence 60% • Response with subsequent failure 5% • No response 30-40% –BMT –IL-3 Transient Erythroblastopenia of Childhood (TEC) • Gradual onset of pallor in previously healthy children age 1-4 years (85%) • Normochromic-Normocytic anemia with reticulocytopenia • Marrow erythroid hypoplasia, aplasia, or recovery • Spontaneous recovery in 4-8 weeks • May have transient neurological features or neutropenia Pathogenesis of TEC • More than half of patients with TEC have viral illness in preceding 2 months • No single viral etiology – Parvovirus leading theoretical candidate – Parvovirus IgM positive in ¢ 12% of cases – Parvovirus DNA detected in 0/16 cases of TEC2 • Several studies1 have demonstrated inhibitory effect of TEC serum and IgG on erythroid colony growth . 1 Freedman, MH Br J Hem. 1993 (85): 246-253 2 Rogers BB, Ped Path Lab Med1996;16(3):471-8 Pathogenesis of TEC • “Thus, TEC is likely to have an immune aetiology. The transient nature of TEC is similar to other autoimmune haemotological disorders of childhood such as idiopathic thrombocytopenic purpura and autoimmune hemolytic anemia.” Freedman, MH Br J Hem. 1993 (85): 246-253 Parvovirus and PRCA • Pathophysiology – Small, non-enveloped, single stranded DNA virus – Transmitted by respiratory tract or blood products – Attacks erythroid progenitor cell via the cellular receptor globoside, aka P antigens • Pathology – Gigantoproerythoblasts in bone marrow – appear toward end of aplastic phase • Fig2.Typical gigantoproerythroblasts in parvovirus B19-associated PRCA. (A) Bone marrow histology with several gigantoproerythroblasts (marked by asterisks) that resemble Hodgkin cells. A small regressive erythroblast (marked with an arrow) typically contains the immunoreactive B19-antigen. (B) Bone marrow cytology of another patient with B19 infection showing two gigantoproerythroblasts (marked by asterisks). (Fig 2A, magnification 750; Fig 2B, magnification 400). • Fisch, Paul, Handgretinger, Rupert & Schaefer, Hans-Eckart Pure red cell aplasia. British Journal of Haematology 111 (4), 1010-1022. Parvovirus PRCA - Clinical Syndromes • Healthy volunteers – 5-10 days of erythroid hypoplasia – No significant anemia with normal RBC lifespan • Transient Aplastic Crisis (TAC) in patients with shortened red cell survival – Sickle cell disease, Thalassemia, Hereditary Spherocytosis, Auto-immune Hemolytic Anemia – Resolves in 1-2 weeks as antibodies against parvovirus produced Parvovirus PRCA - Clinical Syndromes • Prolonged PRCA in immunocompromised hosts – Congenital immunodeficiency –HIV – Leukemia patients on chemotherapy • May be successfully treated with IVIG PRCA in patient ZH • DBA unlikely •TEC • Transient aplastic crisis with underlying red cell abnormality, but – Initial peripheral smear, RBC indices normal – Peripheral smear from mother normal – No evidence of active hemolysis at presentation Auto-Immune Hemolytic Anemia (AIHA) Classification of AIHA • Primary – Warm-reactive autoantibodies, usually IgG – Paroxysmal cold hemoglobinuria, usually IgG – Cold-agglutinin disease, usually IgM • Secondary – Identified infection – Auto-immune disease – Malignancy – Drug-induced Diagnosis of AIHA • Clinical Features – Typically present with signs and symptoms of anemia • Pallor • Fatigue • Weakness – Occasionally present with jaundice – May have intravascular hemolysis with hemoglobinuria Laboratory Findings in AIHA • Anemia with normal WBC and platelets • MCV usually not helpful • Peripheral smear – Spherocytes – Polychromasia • Typically have reticulocytosis – Reticulocytopenia well described (up to 20%) – Autoantibody may react with reticulocytes in marrow Direct Antiglobulin Test (Coombs) (From Foerster J. In: Lee G, et al, editors. Wintrobe's Clinical Hematology, ed 9. Philadelphia: Lea & Febiger, 1993, Ch 40.) DAT (Coombs) • Red Cells collected in EDTA, washed • Polyspecific anti-human sera added, centrifuged to enhance agglutination • Cells resuspended, agglutination noted • Positive tests repeated with specific anti- IgG and anti-C3 sera (anti-IgM, IgA available in research labs) Warm Reacting AIHA • 50-70% of AIHA Cases • Usually preceded by viral infection in children • Onset after infection may be acute, but usually short-lived • RBC destroyed in spleen (extravascular hemolysis) • Usually respond to steroids Warm AIHA Lab Features • Usually IgG antibody • DAT positive for IgG +/- C3 – IgG only 20-66% – IgG and C3 24-63% – C3 only 7-14% • Indirect AT positive in 80% • Antibody reacts at 37º, fixes complement • Antibody panspecific, typically binds Rh • Usually produces difficulty with crossmatch Cold-Agglutinin Syndrome • Typically produces chronic mild hemolytic anemia exacerbated by cold exposure in older adults • Transient secondary form is associated with Mycoplasma infection in adolescents, young adults – symptoms appear 2-3 weeks after infection starts – resolves spontaneously in 2-3 weeks – also reported with CMV,VZV,EBV,Rubella Cold Agglutinins: Laboratory Features • Typically IgM antibody, reacts at 4º • Antibody reacts to polysaccharide antigens, especially I/i group • DAT positive for C3 only • IgM fixes complement efficiently, causing intravascular hemolysis Paroxysmal Cold Hemoglobinuria • Most cases occur in children after infection – Viral - measles, mumps, EBV,CMV, VZV, adenovirus, influenza A – Bacterial - Mycoplasma, H. influenzae, E. coli • Causes acute intravascular hemolysis – High fever – Hemoglobinuria – Exposure to cold temperature often identified retrospectively (cold water for fever) • Symptoms typically resolve in hours Paroxysmal Cold Hemoglobinuria: Laboratory Features • Caused by biphasic IgG antibody (Donath- Landsteiner Ab) – Bind RBCs efficiently at 0-4º, fixes complement C1, Ab dissociates with warming – Other complement components bind and cause lysis at higher temperatures – Selective for P antigen • DAT positive for C3 only unless performed at cold temperature Mixed-Type AIHA • Patients with Warm-type AIHA may also have a cold agglutinin • Most of these cold antibodies are not clinically significant AIHA in Patient ZH • DAT initially positive for C3 only, later IgG and C3, weak in both cases • Differential Dx – Warm Antibody AIHA with low IgG titer – Mixed Type AIHA – PCH with high thermal amplitude ZH Revisited • Underlying Red Cell Disorder with TAC – Would not explain positive DAT • Pre-existing AIHA with superimposed TAC • New onset AIHA with severe reticulocytopenia – No significant evidence of hemolysis at presentation • Viral illness causing simultaneous AIHA and PRCA
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