Exam 2 Review Hematology

A toddler was brought to the ER due to pain on his right abdomen. He has suffered frequent infections since he was one month old. His familial history indicated his brother and a cousin on his mother’s side also had chronic infections and one died from chronic pneumonia. Ultrasound examination showed enlarged lymph nodes and liver. Lab tests showed WBC of 33x10⁹/L, normal platelet counts, and Hct of 27%. Serum immunolglobulins and complement components were moderately increased. Neutrophilia showed moderate toxic granulation and vacuolation; normal neutrophilic migration and phagocytosis; absence of oxidative metabolism in response to neutrophilic stimulation; no post-phagocytic increase in oxygen consumption; absence of formation of superoxide anion and hydrogen peroxide, and the inability of neutrophils to oxidize and kill the phagocytized pathogens. What is the most probable diagnosis?

A 69 yr old male presented hepatosplenomegaly. The cell morphology indicated marked RBC abnormalities, marked teardrop-shaped RBCs (dacrocytes), circulating erythroblasts, immature granulocytes; atypical platelets, micromegakyocytes, giant platelets. The BM was fibrotic. Increase LAP. Cytogenics showed negative for Philly chromosome but JAK2 mutation positive. What is the most likely hematological disorder?

A 5 yr old child presented a 6 week history of gingivitis. He had no serious illness. H had frequent pimples and tended to scratch his skin. Recently, he developed mouth ulcers. His gum bled easily when brushing his teeth. He tanned easily in the sun w/out burning. There were bruises on his limbs but no purpura. He had palpable spleen and some enlarged lymph nodes in the anterior cervical area. His WBCs and other granule-containing cells showed large gray-brown granules of irregular shape and abnormally large peroxidase-positive lysosomal granules. What is the most likely hematological disorder?

A 72 yr old female presented ruby completxion, weight loss, fatigue, weakness, and low grade fever. She also complained of headache, light-headedness, blurred vision, and visual disturbances. Her spleen was enlarged. Her CBC showed WBC of 12.1x10³ /uL, RBC: 6.2x10⁶ /uL, Hct of 55.8%. Platelets: 494x10³ /uL. Further testing showed normal arterial oxygen saturation. Increase LAP. Cytogenetics showed negative for Philly chromosome but JAK2 mutation positive. What is the most likely hematological disorder?

A 38 yr old female presented no symptoms and recently had a routine doctor visit. His CBC results showed WBC: 5.2x10³ /uL, RBC: 3.5x10⁶ /uL, Hct: 32.1%, and Platelets: 794x10³ /uL. The physician order cytogenetics, which showed negative for Philly chromosome and JAK2 mutation. What is the most likely hematological disorder?

An 18 yr old male patient presented a mediastinal mass w/ a high WBC of 110x109 /L. His liver and spleen are moderately tender. Lab tests showed 60% blasts. Flow cytometry showed positive for CD3, 7, 34, 45, and TdT. Surface immunoglobins are negative. What is the most likely diagnosis?

A 52 yr old male w/ a WBC count of 151x103 /uL, 1% promyelocytes, 15% myelocytes, 20% metamyelocytes, 5% basophils, 2% blasts, 20% bands, 20% segmented neutrophils, 1% eosinophils, 10% lymphs, 6% monocytes. He presented a sore throat and an ulcer in his mouth. Anemia and thrombocytopenia are also present. Cytogenetics showed positive for t(9;22). Decrease LAP. Flow cytometry showed positive for CD13, 33. What is the most likely hematological disorder?

A 5 yr old female presented some developmental delays but her cognitive and motor skills to be within normal limits for his age. The manual differential showed granulocytes w/ prominent granules (similar to toxic granulation) in the cytoplasm of segmented neutrophils, lymphocytes and monocytes. Special tests indicated a deficiency of lysosomal enzymes that degrade mucopolysaccharides (lipids) (or glycoaminoglycans). What is the most likely hematological disorder?

A 10 y/o child w/ fever and lymphadenopathy in the region cervical lymph nodes. Flow cytometry showed CD19+, CD10+, most CD34+, CD45 neg. Cytogenetics showed blasts contain >50 chromosomes. What is the most likely diagnosis?

A 35 y/o male came to ER complaining marked prostration and general malaise, pallor, bruising, bleeding, palpitation. CBC showed thrombocytopenia and anemia. WBC differential showed basophilia and dysplasia in granulocytes and erythroid. Flow cytometry showed MPO, CD13, 33, 38, HLA-DR, CD117, 34, and 15. Cytogenetics indicated positive for oncogene DEK-NUP214. What is the most likely diagnosis?

A newborn w/ Down syndrome had a CBC that showed WBC of 46x10³ /uL, platelet count 72x10³ /uL, and 26% blasts. Immunophenotype indicated positive for CD34, 56, 117, 13, 33, 7, 4 dim, 41, 42, 36, 61, and 71. Cytogenetics indicated trisomy 21. A month later, the baby’s CBC was relatively normal. What is the most probable diagnosis?

A 69 y/o male presented hepatosplenomegaly. WBC count was 32x10⁹ /L. WBC differential showed neutrophilic granulocyte proliferation, most PMNs and bands with toxic granulation and coarse granules, <10% myelocytes, metamyelocytes and are promyelocytes. Cytogenetics chowed normal cytogenetics and negative for Philly chromosomes. What is the most probable diagnosis?

A CBC of a 58 y/o woman revealed a relative normal CBC except for the platelet count of 100x10³ /uL. Peripheral smear showed large and giant platelets and pale blue inclusions resembling Dohle bodies in granulocytes; giant platelets.

STEPS OF PHAGOCYTIC ACTION BY NEUTROPHILS IN ORDER

1-5

The min. blast percentage to be considered acute myeloid leukemia in the WHO system is: