blood Pediatric Hematology Articles

Apr 27, 2017
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An 18-month-old girl with a diagnosis of Chediak-Higashi syndrome (CHS) presented with severe bacterial skin infection. Recurrent episodes of bacterial infections began at the age of 2 months. On physical examination, generalized lymphadenopathy, hepatosplenomegaly, skin hypopigmentation, and silvery hair were noted. Bone marrow aspirate revealed prominent granules (top left and right) within the lymphocytes and myeloid cells. Phagocytosis of red cells and red-cell precursors (arrows) is also seen. A diagnosis of “accelerated phase” of CHS was made. She underwent an unrelated allogeneic hematopoietic stem cell transplantation. Three years later she is well and attending school. Chediak-Higashi syndrome is a rare childhood autosomal recessive disorder of defective phagolysosome dysfunction. The gene CHS/LYST1 on chromosome 1q42-43 codes for CHS/LYST1 protein that is associated with microtubules and regulates lysosome-related organelle size and movement.

A 22-year-old man presented with longstanding history of pallor and intermittent transfusion support since the age of 10. Family history was significant with his elder brother having longstanding history of transfusion-dependent anemia, and his parents had consanguineous marriage. Examination revealed pallor, icterus, splenomegaly, and severe gouty tophi leading to deformity of fingers of the right hand (top right) and an ulceration of a toe (bottom right). Complete blood count showed hemoglobin, 53 g/L; hematocrit 0.17 L/L; MCV 80.2 fL, white cell count 5.9 × 109/L, and platelets 395 × 109/L. On the peripheral blood smear, there was anisocytosis, polychromasia, and numerous nucleated red blood cells. Bone marrow examination (shown on the left) revealed numerous binucleated and multinucleated erythroid precursors, typical of congenital dyserythropoietic anemia type II.

A male neonate was born at 28 weeks of gestational age by C-section because of intrauterine growth restriction and fetal anomalies. Radiographic scans showed cardiomegaly, pleural effusion, and ascites, consistent with hydrops fetalis. He developed a disseminated rash, respiratory failure, anemia, and thrombocytopenia. A complete blood count showed corrected white blood cell count 9.3 × 109/L, hemoglobin 112 g/L, platelets 16 × 109/L, MCV 139.1 fL (normocytic for gestational age), and 353 nucleated red blood cells per 100 white blood cells counted. Morphologic examination of the peripheral blood noted erythroid precursors at every stage of development, as well as a few circulating mitotic figures (see photos). An evaluation for hemolytic disease of the newborn (HDN) was negative. Viral culture of the amniotic fluid was positive for cytomegalovirus (CMV).

A 16-year-old female with AIDS presented with 3 months' history of fever, loose stools, and significant weight loss. She had marked pallor, erythematous plaques on the face and neck, enlarged liver and spleen 2.0 cm, below the respective costal margins, and bilateral cervical lymphadenopathy. Both her parents had been suffering from AIDS and subsequently died of disseminated tuberculosis. Her chest x-ray was normal and ultrasonography affirmed hepatosplenomegaly. HIV serology was positive and CD4 count was 0.012 × 109/L. Complete blood counts showed pancytopenia with hemoglobin of 57 g/L, total leukocyte count of 1.1 × 109/L, and platelet count of 18 × 109/L. The peripheral blood film (panel A) revealed monocytes and neutrophils with the presence of yeast-like intracellular organisms, 2 to 4 µm in diameter.

The patient is a 10 month old Caucasian male with a history of recurrent upper respiratory infections and otitis media since birth. His CBC at this time included a H/H of 10.9g/dl/32.4%, platelet count of 280,000/ul and a WBC of 3840/ul with a differential of 25% monocytes, 70% lymphocytes, 4% eosinphils, 1% basophils and 1% neutrophils. His ANC was 38/ul. After an extensive evaluation he was diagnosed as having congenital neutropenia consistent with Kostmann's syndrome. The peripheral smear confirmed the leukopenia and almost complete absence of neuutrophils. A single hyposegmented PMN can be seen in this view.

Marrow aspirate of a 15 month old child with a history of hypertonicity, aspiration pneumonia and hepatosplenomegaly. A high power view of the cells illustrates the benign-appearing nuclei and the "crumpled tissue paper" appearance of the cytoplasm.

Patient is a 6-year-old child with leishmaniasis who presented with fever and mild splenomegaly. This bone marrow aspirate shows extracellular amastigotes, each containing a nucleus and a bar shaped kinetoplast. Wright-Giemsa stain, original magnification 100× (oil).

Peripheral smear from a patient with sickle cell disease. Multiple RBC abnormalities are noted, including a nucleated RBCs, target cells, microspherocytes (post-transfusion), and Pappenheimer bodies.Additional findings are the presence of target cells (large arrow) and sickle cell (small arrow).

Peripheral smear from a 3-year-old with history of pica who had a hemoglobin of 9.5 g/dL. RBCs are normochromic/normocytic but polychromatophilic cells are markedly hypochromic and contain multiple coarse basophilic granules. (W-G 50x)

Bone marrow aspirate from an infant with ALL has increased numbers of immature lymphoid elements and lymphoblasts.

The RBC deformity (arrow) shown in this image is referred to as a “bite” cell. This abnormality can be seen in when hemoglobin precipitates (Heinz bodies noted on supravital staining) attach to the RBC membrane and are removed when these cells pass through the spleen. Disorders associated with this abnormality include a thalassemia (ß chain tetramers) and G6PD deficiency manifested after oxidant drug exposure.


My Say

Notes from a Hawkish Pediatric Hematologist

Crystal L. Mackall, MD
Chief, Pediatric Oncology Branch
National Cancer Institute
Bethesda, MD

Meet the Editor

Crystal L. Mackall, MD

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