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General Hematology Slide Review • 15 month old with pallor and hemoglobin 2.5g/dl, MCV 62fl and retic 4% • Drinks 48 oz of whole milk/day • What is this child’s problem? Iron Deficiency Anemia • Family lives on a farm and brings in their 2yo for you to examine… • Child is pale and an HPD reveals a hemoglobin 4g/dl, MCV 102fl • You diagnose a macrocytic anemia secondary to what? Folate deficiency Unpasteurized Goat’s Milk • 7 yo boy with: – – – – – fever x 1 week Hemoglobin 9g/dl WBC 1,200 Plt 32K Ferritin 6000 What’s his diagnosis? Hemophagocytic syndrome Hemophagocytic Syndrome • Non-malignant disorder of antigen-presenting macrophages • Caused by: – Uncontrolled hemophagocytosis – Defective NK cell function – Cytokine upregulation • Familial and sporadic forms • Appears in association with: – Bacterial, parasitic or viral (EBV) infections – Malignancy – Drugs – Connective tissue disorders (SLE, Kawasaki’s) • Diagnosis: – – – – Fever, splenomegaly Cytopenias (>2 cell lines) Elevated ferritin, triglyceride Hemophagocytosis in marrow, spleen, lymph nodes • Prompt diagnosis/treatment essential What is this? • 14 month old AfricanAmerican child presents with mild anemia – HPD obtained and when reviewed showed these cells. • What are they? Elliptocytes Hereditary Elliptocytosis • 5 yo African-American boy recently presented with fever. Prescribed Bactrim for presumed otitis media. Brought back in by mom due to increased fatigue and this peripheral smear… What is the cause of this peripheral smear? Glucose 6-Phosphate dehydrogenase (G6PD) deficiency G6PD Deficiency • Jaundice in first 24 hours of life (pathologic jaundice) • G6PD gene located on X chromosome – females can be affected • Common in certain ethnic groups • Newborn in your practice – healthy, no prenatal complications • His newborn screen results comment on a “fast” band seen on hemoglobin separation What’s his diagnosis? Hemoglobin H disease Hemoglobin H Disease • Caused by 3-gene deletion a thal • Excess b chains • Tetramers precipitate forming red cell inclusions • High O2 affinity leading to reduced O2 carrying capacity • 2 yo AAM presents with pain in lower extremities x 2 days • Peripheral smear and hemoglobin separation reveal this… • What’s the diagnosis? Homozygous sickle cell anemia • Same patient presents with h/o fever and marked pallor • Hemoglobin 2.5g/dl • Retic 0.1% • What’s your diagnosis? Aplastic Crisis Parvovirus B19 infection What does this child have? Chédiak-Higashi • • • • Autosomal recessive Absence of pigment in eyes, skin and hair Giant granules in leukocytes Increased susceptibility to infections – Defective movement – Decreased granulation – Decreased microbicidal activity • Frantic doctor refers a child to your practice with a platelet count of 20K. • Child has no bleeding problems and is completely healthy. • What’s your diagnosis? Pseudothrombocytopenia EDTA • Another child presents with epistaxis, “wet” purpura in mouth and bruises/petechiae over trunk and extremities • Platelet count 2K with remainder of counts normal What’s the diagnosis? Immune Thrombocytopenic Purpura