Hematopathology Flashcards
anisocytosis
RBCs are of unequal size
poikilocytosis
abnormally shaped rbc/s in blood
How can erythroblasts be released from a fetus?
Erythroblastosis Fetalis/ Hemolytic Disease of Newborn:
If a mother lacks an antigen expressed by the fetus, maternal IgG alloantibodies can cross the placena causing complement-mediated hemolysis of fetal erythrocytes and release of erythroblasts.
What does idiopathic myelofibrosis feature?
Marrow collagen deposition (fibrosis)
In PV, describe characteristics of the spleen and BM
BM: hypercellular with hyperlasia of all elements
Spleen: slightly enlarged, cut surface is uniformly dark red, with expansion of the red pulp and obliteration of the white pulp
What is rearranged in irreversibly sickled cells?
Phospholipids between the outer and inner monolayers of the cell membrane
What leads to Renal Papillary Necrosis in SCD patients?
low oxygen, low pH, high osmolality in the renal medulla
Ananthocytosis
aka Spur cells
formed from a defect within the lipid bilayer of the red cell membrane and feautres spiny projections of the surface with centrally dense cytoplasms which may be associated with hemolysis.
Can be caused by increased free cholesterol
What is common presentation of MDS?
Anemia, Neutropenia, Thrombocytopenia
Differential for ringed sideroblasts
- Sideroblastic Anemia
- Myelodysplastic Syndrome
What are some risk factors for MDS?
Chemotherapy Radiotion The rap y Viruses Benzene Expsoure Cigarrette Smoking Fanconi Anemia
Clinical symptom of petechiae is related to what lab finding?
thrombocytopenia
What are some causes of injury to bone marrow stem cells?
Idiopathic (2/3)
Toxic (ex. benzene)
Immunologic
Hereditary (Fanconi Sndrome)
Schilling test
Vitamin B12 absorption
which protein is encoded by brc/abl of CML? Childhood ALL?
Fusion protein p210; Fusion protei P190
What is Waldenstrom Macroglobulinemia
Neoplasm of small lymphocytes nad a variable number of IgM-secreting plamsa cells of the same malignant clone (M spike characteristic)
A leukemic blast that tests positive for TdT implies lymphiod or myeloid lineage?
lymphoid lineage
Prefibrotic Stage of Myelofibrosis
Bone marrow is hypercellular , with predominant neutrophilic and megakaryocytic proliferation
Fibrotic Stage of Myelofibrosis
PBS shows either leukopenia or leukocytosis, and myeloid precursors and nucleated RBCs are usually present. Defined by conspicuous reticulin or collage n fibrosis
What does hyperplasia of the secondary follicles, germinal centers, and plasmacytosis of the medullary cords indicate?
B-lymphocyte immunoreactivity
Where is interfollicular of diffuse hyper plasia ?
Hyperplasia of the deep cortex or paracortex
What’s a starry sky macrophage?
appearance of the cellular deris of apoptotic tumor cells cleared by macrophages
What skin pathology can be seen in LCH?
Seborrheic or eczematoid dermatitis
What clinical finding of the ear is associated with LCH?
Otitis Media
What gene arrangement is often seen in follicular lymphoma?
bcl2
How do cold aggulitinins normally develop?
They are idiopathic or secondary to infections (such as EBV) or lymphoproliferative disorders.
immunoglobins warm and cold
IgG or Ig M respectively
What organ systems are affected by TTP?
Renal Impairment and Neurological Symptoms
Hallmark of TTP
Deposition of PAS positive hyaline microthrombi (composed of platelet aggregates, fibrin, and a few RBCs and luekocytes) in arterioles and capillaries
Where do Hemophilia A patients tend to bleed?
Joints, muscles, internal organs
What tests can help diagnose PNH?
Sucrose Hemolysis Test Ham Test (acidified serum) Flow Cytometry (Loss of GPI-anchored proeins on blood cells b)
Genetic Mutations (somatic mustation of phosphatidylinositol glycan-class A- PIG-A)
How does leukocytosis develop in CML?
neoplastic cells in CML are derived from an abnormal pluripotent bone marrow stem cell which results in prominent neutrophilic leukocytosis over the full range of myeloid maturation
what neutrophil count indicates neutrophilia?
above 7000
What indicates a benign Leukomoid Reaction over a CML rxn?
- cells in PBS are more mature than myelocytes
- Leukocyte alkaline phosphatase activity is high
- Large blue cytoplasmic inclusions known as “Dohle Bodies” or toxic granulation is present.
What epithelial cell disease is associated with Myasthenia Gravis?
Thymoma (Epithelial cells of the thymus)
PTLD is associated with what disease?
EBV INFECTOIN
What is a Russel Body?
plasmacytoid lymphocytes that have stored immunoglobulins in the cytoplasm.
In Polycythemia Vera, what could lead to pruritis and peptic ulceration?
Histamine release from basophils
PV can burn out, blast out, and develops into what 3 diseases?
Myelofibrosis, AML, OR CML
What are some causes of splenic enlargement?
Amyloid Deposition
Congestive Splenomegaly (from Portal HTN or Cirrhosis)
Storage Diseases (such as Gaucher Disease)/Metabolism Disease (Galactosemia)
Myeloproliferative Disorders
AML
T-cell hyperplasia in the PALS (seen in IM)
What do Auer rods consist of?
Condensations of azurophilic granules
What does a Dohle body consist of?
Patches of Dilated Endoplasmic Reticulum
What is a toxic granulation?
Course and dark primary granules that are reactive changes in mature neutrophils most indicative of marked inflammation, such as bacterial sepsis.
What does a Heinz body consists of?
precipitates of denatured globin as seen in G6PD deficiency
What cytogenetic and molecular analysis is expected in CLL?
In this Clonal B-cell neoplasm , there is a rearrangement of immunoglobulin genes and T-cell receptor genes are in germline configuration
What lymphocyte is elevated in SLE?
Monocytes (Monocytosis)
Infection from what organism results in lymphadenopathy with microscopic stellate necrosis?
R. henselae (in Cat-Scratch Disease)
Infection from what organism results in acute lymphadenopathy with microscopic hemorrhagic necrosis?
Y. Pestis (causes Plague)
What is the effect of AML on the spleen? CML?
AML: enlargement of the spleen (with uniform infiltration of the parenchyma- no focal lesions)
CML: enlargement of the spleen ( with risk of splenic infarcts)
Describe the appearance of a lymph node draining from a coancer that shows a reactive pattern
Dilated Sinusoids with endothelial hypertrophy
Filled with histocytes (ex. macrophages)
(Sinus histocytosis represents an immunolgic response to cancer antigens)
CD 68
MACROPHAGE (HISTIOCYTE )MARKER`
CD 11c
MONOCYTE marker
Describe a Hairy Cell
leukocyte with reniform nuclei and pale blue cytoplasm with threadlike extensions. Leukemic hairy cells are B cells
CD 3- CD56+
NK cells
CD19+, SiG+
Mature B cells
CD33+ CD13+
monocytes/granulocytes
NOTCH 1 gene is associated with what lymphocyte?
T cell. It encodes for a transmembrane receptor required for T cell development. more than half of pre-T cell tumors have activating point mutations.
Nodular sclerosis type HL is more common in what patient population?
women
Anaplastic Large-cell Lymphoma is most often characterized by what gene arrangement?
Gene arrangement on chromosome 2p23 that results in production of anaplastic lymphoma kinase (ALK) with tyrosine kinase activity.
the c-KIT protooncogene has been associated with which NK cell lymphomas?
NK cell lymphomas
RS of HL have what gene rearrangement and what cell oirgin?
RS cells contain the EBV genome and are derived from B cells. All RS cells have clonal (identical) immunoglobulin gene rearrangements.
In Cat Scratch Disease, where is lymphadenitis often seen?
“Upstream” of lymphatic drainage from the site of injury, so that the axillary and cervical lymph nodes are most often involved
deletions of 5q is often seen in what syndrome?
myelodysplastic syndromes
