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Hematopoietic and Lymphoid Systems Grace C. Tenorio, M.D. February 7, 2007. Red Blood Cell Disorders. Anemias Anemia of Blood Loss: Acute vs. Chronic Hemolytic Anemias Hereditary Spherocytosis Sickle Cell Disease Thalassemia Paroxysmal Nocturnal Hemoglobinuria
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↑ erythropoiesis: reticulocytosis
Iron accumulation: hemosiderosis
Intravascular: heat, clostridia toxin, trauma complement fixation
hemoglobinemia, hemoglobinuria, hemosiderinuria
No hemoglobinemia or hemoglobinuria
- Autoimmune, drug-induced
- Antigen that stimulates antibody or complement- mediated destruction of RBCs
1. Warm antibody: idiopathic, B-cell Neoplasms (CLL/SLL), SLE, drugs (aldomet, PCN, Quinidine)
2. Cold antibody: M. pneumoniae. Infectious mono.
- + DAT detects anti-RBC antibodies
- Microspherocytes on peripheral smear
Management: Treat underlying disorder, FFP/ CRYO, ?? heparin, recombinant activated protein C (Xigris)
1. Splenic sequestration
2. ↓ Production: BM failure (AA, Rx, tumor)
3. ↑ Destruction (N or ↑ megakaryocytes in BM)
Example: HIV = suppression of megas, immune complex mediated injury of PLTs, autoantibodies
A 43 year old woman presented to her physician with the complaint of easy bruising. Physical examination showed multiple petechiae and a large ecchymotic area in the thigh following minor trauma
Lazarchick, J. ASH Image Bank 2001;2001:100177
Acute self-limited disease of adolescents/young adults
X-linked lymphoproliferative disorder-inability to mount an immune
response to EBV, boys with SH2D1A gene mutation.
Acute self-limited lymphadenitis of childhood (<18 y/o)
* sIg – surface immunoglobulin **cIg – cytoplasmic immunoglobulin
“starry sky pattern”
1. ↓ in normal RBCs, WBCs and PLTs
2. Aim of TX is to reduce the leukemic clone to allow reconstitution with the progeny of remaining normal stem cells
Ph chromosome - poor
1.Clonal plasma cell (myeloma cells) proliferation (BM)
2. Lytic bone lesions
MDS and CMPD transform to AML
Treatment: Gleevec (Imatinib®)
Tyrosine kinase that targets BCR-ABL fusion protein
Langerhans cell histiocytosis
“HSC triad”- exophthalmos, diabetes insipidus, calvarial bone lesions