Heme PANCE

IDA

blood loss

Hct < 25%

• Nl initially
• Later hypochromic microcytic red cells, anisocytosis, and poikilocytosis

only one alpha chain present

B thall major

 problems begin at 4-6 months, when the switch from fetal Hgb to adult Hgb

Serum iron and ferritin levels are nl

Hgb elcectrophesis

• folic acid supplements, avoid iron suppleemnts and oxidative meds
• B major: transfusions

Deferoxamine

hypochromic

Prussian Blue staining

myelodysplasia, chronic alcoholism, and lead poisoning

Te cell meidate autoimmune suppression of hematopoesis

weakness, fatigue, vulnerability to infection, pallor, purpura, and petechiae

pancytopenia

normochromic normocytic

poor dietary intake

Phenytoin, Bactrim, sulfasalazine

50-100 mg

Pregnancy, hemolytic anemias, and exfoliative skin dz

Macro-ovalocytes and hypersegmented PMNs

macroovalocytes, hypersegmented PMN, Howell-Jolly bodies

nuclear DNA remnants

1 mg/day

pernicios anemia

terminal ileum, storage in the liver

• glossitis, pale icterus, vague GI sx
• neur: stocking-glove paresthesias, loss of position, fine touch and vibratory sensation, clumsiness, dementia, and ataxia

anisocytosis, poikilocytosis, macro-ovalocytosis, and hypersegmented neutrophils

Reduced reticulocyte count, elevated LDH and indirect bilirubin, low B12

lifelong Im injections for pernicious anemia, daily oral cobalmin in high doses

Treated within 6 months

hereditary spherocytosis and elliptocytosis, G6PD, methemoglobinemia, SCA

autoimmune and lymphoproliferative diseases, drug toxicity, TTP, HUS, DIC, valvular hemolysis, metastatic adenocarcinoma, vasculitides, infections, hypersplenism, burns

jaundice, gallstones, pallor, and sx

Parvovirus

elevated reticulocyte count in presence of falling or stable HCT

immature red cells, nucleated red cells, nucleated red cells, or morphologic changes

transient hemoglobinemia, elevated unconjugated bili, serum LDH high (microangiopathic hemolysis)

autosomal recessive

RBCs containing primarily HGB S sickle under deoxygenated conditions

red cell dehydration, acidosis, hypoxemia, high altitudes

cholelithiasis, splenomegaly, poorly healing ulcers, infection with encapsulated organisms, strokes, priapism, retinopathies, osteomyelities, avascular necrosis

HGB electrophoresis, PBS

sickled cells and target cells, nucleated RBCs, HJ bodes

• elevated tic count
• Elevated wbC count
• thrombocytosis may be present
• indirect bili may be elevated

• Analgesics, fluids, oxygen
• transfusions for vaso-occlusive crises
• Pneumococcal vaccine q10 years, folate supplementation

x-linked recessive disorder commonly seen in American black males and some mediterreanean populations

ASA, dapsone, primaquine, quinidine, sulfonamides, nitrofurantoin)

• tic count and serum indirect bili increase during hemolytic episodes
• PBS reveal bite cells and Heinz bodies
• G6PD levels low

bite cells and Heinz bodies

• self-limited as RBCs are replaced
• avoid oxidative drugs

• slowly progressive bone marrow disorder characterized by increased numbers of RBCs and increased total blood volume;
• unregulated expansion of RBC causes hyperviscosity, which leads to decreased cerebral blood flow

presence of JAK2 mutation

thrombosis

M/F, median age of presentation 60%

11-15 years

myelofibrosis or CML, rarely to AML

splenomegaly, nl arterial O2 sat, elevated RBC mass

sx of increased blood viscosity (HA, dizziness, fullness in head and face, weakness, fatigue, tinnitus, blurred vision); burning, pain, redness of extremities, stroke

PCV

• epistaxis (may be presenting sx)
• hPUD
• plethora, systolic HTN, engorged retinal veins, splenomegaly

• HCT > 54% males, 51% females
• PBS shows neutrophilic leukocytosis, increased basophils and eosinophils, increased numbers of large, bizarre plts
• nl RBC morphology
• hypercellular bone marrow in all cell lines
• hyperuricemia

neutrophilic leukocytosis, increased basophils and eosinophils, increased numbers of large, bizarre plts

• phlebotomy is tx of choice
• myelosuppressive therapy with hydroxyurea may be indicated; anagrelide may be added or substituted
• low dose ASA reduces risk of thrombosis without increasing the risk of bleeding

diseases characterized by unrestrained growth of leukocytes and leukocyte precursors in the tissues

ALL

3-7 yo

avg age of onset 60 yo

• gingival bleeding, epistaxis, menorrhagia; DIC is less common
• fatigue, abrupt onset of fever, lethargy, HA, and bone and or jt pain (esp in sternum, tibia, and femur)

pancytopenia with circulating blasts; blasts make up at least 20% of nucleated cells in bone marrow

• hyperuricemia
• pancytopenia with circulating blasts
• Auer rods in AML
• high WBC

AML

ALL

ALL

clonal malignancy of B lypmhocytes

CLL

B cell form

prognostic staging for CLL

young to middle aged adults (mediat age is 55 years)

chronic, accelerated, and acute (aka blast crisis, >30% blasts)

• fatigue, anorexia, weight loss, low-grade fever, excessive sweating
• abdominal fullness caused by splenomegaly
• runs a mild course until blast phase

• peripheral lymphocytosis and lymphotic invasion of bone marrow, liver, spleen, and LNs
• recurrent infections, HSM

a type of CLL, where an isolated node transforms into aggressive, large cell lymphoma

isolated lymphocytosis (leukocytosis > 20,000 cells)

leukocytosis, with meadian WBC count of 150,000

gene used to idenitfy chronic leukemia

anemia and thrombocytosis

mature small lymphocytes, smudge cells

CLL

• Gleevec, dastinib or nilonib in cases of Gleevec intolerance
• allogenic bone marrow transplant may bt eh initial tx and is the only therapy proven to be curative

• chemotherapy, consolidation therapy
• bone marrow transplant if poor reponse to tx

pallaitive once dz is advanced

group of cancers characterized by enlargement of lymphoid tissue, spleen, and liver and the presence of reed-sternberg cells

Hodgkin's Disease

EBV

15-45 yo, peaking in 20s, and again after 50; rare under 5 yo

painless cervical, supraclavicular, and mediastinal lymphadenopathy; pain in affected node after ingestion of alcohol

lack of constituational sx

presence of constitutional sx

Staging system for Hodgkins and NHL

• combo chemo
• radiation therapy nitial tx of choice for pts with low risk stage IA and IIA dz
• most other pts receive adriamycin, bleomycin, vinblastine, and dacarbazine chemo (ABVD)

group of malignancies that arise from lymphocytes, usually from B lymphocites

Peak incidence between 20 and 40 yo, lymphomas divided into clinically indolent and addgressive groups

diffuse or isolated, painless, persistant lymphadenopathy is the m/c presentation, extralymphatic presentation, some constitutaionl sx

diffuse or isolated, painless, persistant lymphadenopathy

GI tract, skin, bone, and bone marrow

abdominal fullness

Serum LDH

• some spontaneous remission
• single involved node: radiation
• low grade lymphoma: rituximab with or without chemo
• aggressive: Low grade lymphoma=allogenic transplant
• Intermediate or high grade lymphomas: chemo and autlogous stem cel transplant

• malignancy of plasma cells, possibly caused by HPV
• replacement of bone marrow leads to failure, bone destruction leads to pain, OP, lytic lesions, hypercalcemia, and pathologic features

Neutropenia and failure of Ab production in reponse to antigen challenge

65 yo

• anemia, bone pain (particularly in low back or ribs, and infection)
• also renal failure, spinal cord comperssion, and hyperviscosity syndrome

MM

MM

MM

lenalidomide, dexamethasone, and doxorubicin; with bisphosphonates as adjuncts

rate of conversion between fibrinogen and fibrin in the presence of thrombin

TCP

impaired production, increased destruction, splenic sequestration, or dilution

self limited, auto-immune disorder found commonly in children of both sexes and is associated with a preceeding viral URI

ITP

abrupt appearance of petechaie, purpura, and hemorrhagic bullae

petechiae on skin and mucous membranes

Heparin

sufulonamides, quinine, thiazides, cimetidine, and gold

• decreasd plalets (b/w 10-20k), eosinophilia, and mild lymphocytosis
• mild anemia, nl coag

• plts b/w 25-75k
• mild anemia, nl coag

usually resolves spontaneously, some pts require corticosteroids or splenectomy

• high dose prednisone
• If tx fails: IVIG, danazol, immunosuppresive therapy, stem cell transplant

TTP, HUS, and DIC

20-50 yo, m/c in women than men, common in HIV

Estrogen use, pregnancy, drugs such as quinine and ticlopidine

Abs against ADAMTS13 enzyme, which breaks down large multimers of VWF. therefore an increase in VWF leads to an increase in clotting at sites of vascular injury

generalized hemorrhages in pts with severe underlying systemic illness such as sepsis, tissue injury, obstetric complications, cancer, and severe transfusion reactions

• purpura, microangiopathic hemolytic anemia, fever, abnl neurologic signs, renal failure
• petechiae, apllor, abdominal pain, possibly pancreatitis

Child version of TTP without neurologic findings, but with more kidney problems

<10 yo, particularly after infection with E coli, shigella, salmonella, and various viruses

skin and mucous membrane bleeding (particularly at puncture/wound sites), shock, thrombosis

• anemia, red cell fragmentation (Schistocytes), nl leukocytes, polychromaphilia, reticulocytosis, and TCP (less severe in HUS than in TTP), negative Coombs
• High LDH
• High indirect bili
• nl coag
• renal insufficienc

• anemia, red cell fragmentation (Schistocytes), nl leukocytes,
• polychromaphilia, reticulocytosis, and TCP (less severe in HUS than in
• TTP), negative Coombs
• High LDH
• High indirect bili
• nl coag
• renal insufficienc

• Evidence of coagulopathy (hypofibrinogenmia, elevated fibrin degradation products)
• TCP
• prolonged PT
• Absent ADAMTS13
• Microangiopathic hemolytic anemia with schistocytes

fragmented RBCs

• large volume plasmapheresis
• prednisone and antiplatelt agents

• conservative management
• fluids and management of electrolyte imbalance
• in adults, plasmapheresis

• tx underlying cause
• component blood transfusions are important; role of heparin is controversial

NSAIDS, ASA

autosomal dominant

vWD

• bleeding in nasal, sinus, vaginal, and GI mucous membranes
• rare bleeding into joint spaces

• nl pt/ptt, prolonged bleeding time
• low vwf

• vasopressin for type I
• factor VIII concentrates are preferred if factor replacement is necessary

Hemophilia A

a hereditary disease caused by excessively prolonged coagulation time

x linked recessive

hem a

• Spontaneous hemorrhagic episodes with hemearthroses, epistaxis, intracranial bleeding, hematemesies, melena, microscpoic hematuria, bleeding into soft tissue and gingiva
• excessive bleeding following trauma/surgery

• Prolonged PTT
• PT, bleeding time, fibrinogen level, plts nl
• reduced factor 8, vwf nl

• infusion of heat treater or recombinant factor 8
• desmopressin may elevated levels in mild to mod dz
• avoid ASA, use celecoxib or opiods for pain

Hem B

Hem B

X linked recessive disorder

found in Ashkenazi Jews

Vit K

• poor diet
• liver failure
• malabsorption
• malnutrition
• use of some drugs

• PT prolonged, PTT may be prolonged
• Fibrinogen, thrombin time, plt count are nl
• liver enzymes may be elevated

• Tx underlying cause
• Vit K (PO or IV)
• tx hemrorhage with FFP

2, 7, 9, 10

Acquired hypercoaguable states associated with malignancy